Diagnostic Criteria In Dermatology

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Atopic Dermatitis

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Atopic dermatitis is a common, chronic, pruritic skin condition associated with increased sensitivity of the skin and mucous membranes to external antigens (atopy), increased IgE synthesis, cosinophilia, and an imbalance in cytokines secreted byT-helper I and 2 cells. The disease usually onsets in infancy and may persist throughout life. In adults with a new eruption, it is important to rule out other skin diseases before making the diagnosis, since onset in adulthood is uncommon. In 1933, Wise and Sulzberger first named the disorder “atopic dermatitis” because of its close association with respiratory allergy.

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ATOPIC DERMATITIS
United Kingdom (U.K.) Working Party Criteria (1994)

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Diagnosis requires:
History of an itchy skin condition (or parental report of scratching/rubbing by the child)
PLUS > 3 of:
1. History of involvement of skin creases (antecubital, popliteal, anterior ankles, or around the neck) or cheeks in children < age 10 years
2. Personal history of asthma or hay fever (or history of atopic disease iii St degree relative if < age 4 years)
3. History of generalized dry skin in the past year
4. Visible flexural dermatitis (or eczema on the cheeks/forehead and outer limbs in children < age 4 years)
5. Onset under the age of 2 (do not use as criterion if patient is < age 4 years)
N.B. • SN:69-80%. • SP:93-97%.
• A formal validation study of the U.K. Working Party criteria in infants is still required.
• An  IgE level has SN: 80% & SP: 96%; however, it is invasive, expensive, and not readily available.

Front 3

ATOPIC DERMATITIS
Hanifin & Rajka Atopic Dermatitis Criteria (1980)’
(original criteria)

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Diagnosis requires:
>3 major criteria PLUS >3 minor criteria

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Major Criteria:
1. Pruritus
2. Typical Morphology & Distribution -->Adults: flexural lichenffication or linearity; Infants & children: face and extensor involvement
3. Chronic or relapsing dermatitis
4. Personal or family history of atopy (atopic dermatitis, asthma, allergic rhinitis)

Back 4

Minor Criteria:*
Features of the ”atopic facies”
I. Facial pallor or erythema 2. Pityriasis alba
3. Orbital darkening 4. Dennie-Morgan infraorhital fold
5. Cheihtis 6. Recurrent conjunctivitis
7. Keratoconus 8. Anterior subcapsular cataracts
9. Anterior neck folds
Others:
10. Early age of onset 11. Perifofficuiar accentuation
12. Xerosis 13. Ichthyosis/almar hyperlinearity/eratosis pilaris
14. Non-specific hand or foot dermatitis
15. Nipple eczema
16. White dermatographism/delayed blanch
17. Immediate type I skin test reactivity
18. Elevated serum IgE
19. Tendency toward cutaneous infections (esp. Staph aureus and herpes simplex)/ impaired cell-mediated immunity
Triggers of atopic dermatitis:
20. Food intolerance
21. Course influenced by environmental/emofionál fkEtors
22. Intolerance to wool and lipid solvents
23. Itch when sweating
*The minor criteria have been regrouped for easier reference.

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ATOPIC DERMATITIS
Rajka & Langeland Atopic Dermatitis Grading (1989)

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Severity below requires the following Score
Mild 3-4 ; Moderate 4.5-7.5; Severe 8-9
Area Score*
<9% body surface area
9-36% body surface area 2
>36% body surface area 3
Course
> 3 months remission** during a year
<3 months remission** during a year 2
Continuous course 3
Pruritus severity
Mild pruritus, rarely disturbing night sleep 1
Pruritus > score of 1, hut < score of 3 2
Severe pruritus, usually disturbing night sleep 3
* May use half scores (i.e. 1.5, 2.5)
** No inflammatory lesions; no further treatment required; may have dry skin

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Basal Cell Carcinoma Nevus Syndrome (Gorlin Syndrome)

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Basal cell carcinoma nevus syndrome or ncvoid basal cell carcinoma syndrome is an autosomal dominant condition predominantly affecting Caucasians and caused by mutations in the PTCH gene on 9q2 2.3.’ It was described by American oral pathologist and geneticist Robert Gorlin and American dermatologist Robert Goltz in 1960

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Evans et al Criteria (I993)
Diagnosis requires: 2 Major or I Major PLUS 2 Minor Criteria
Major Criteria:
1. > 2 BCCs or 1 if< age 30 years, or> 10 basal cell nevi
2. Any odontogenic keratocyst (histologically proven), or polyostotic (multiple bones affected) bone cysts
3. > 3 Palmar or plantar pits
4. Ectopic calcffication: lamellar or early (< age 20 years) falx calcification
5. Family Hx of basal cell nevus syndrome

Back 7

Minor Criteria:
I. Congenital skeletal anomaly: bifid, fused, splayed or missing rib; or bifid, wedged, or fused vertebra
2. Occipital-.Frontal Circumference (OFC) > 97 percentile, with frontal bossing
3. Fibromas: cardiac or ovarian
4. Medulloblastoma
5. Lymphomesenteric cysts
6. Congenital malformation: cleft lip and/or palate, polydactyly, or eye anomaly (cataract, coloboma, microphthalmia)

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Behçet’s Disease

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In 1937, aTurkish dermatologist, Hulusi Behçet, described a triple sign syndrome (recurrent oral and genital aphthous ulcers and recurrent eye ulcers) based on findings in 3 patients.’ Three different criteria have been developed in order to make the diagnosis. The most recent criteria are presented first

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International Study Group for Behçet’s Disease Criteria (1989)
Diagnosis requires:
Recurrent oral ulceration: minor aphthous, major aphthous, or herpetiform ulceration observed by physician or by a reliable patient, with recurrence of 3 episodes in one I 2-month period.

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PLUS > 2/4 of the following:
I. Recurrent genital ulceration:
• aphthous ulceration or scarring, observed by physician or by a reliable patient
2. Eye lesions:
• anterior uveitis, or
• posterior uveits or
• cells in vitreous on slit lamp examination or
• retinal vasculitis observed by an ophthalmologist
3. Skin lesions:
• erythema nodosum, observed by physician or by a reliable patient or
• pseudofolliculitis, or
• papulopustular lesions; or
• acneiform nodules, observed by physician in post-adolescent patients not receiving corticosteroids
4. Positive pathergy test read by a physician at 24-48 hours:
• an indurated erythematous papulc or pustule that is> 2mm at the prick site after the application of a sterile needle or intradermal injection of saline
N.B.
• Findings applicable only in absence of other clinical explanations.

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O’Duffy Behçet’s Disease Criteria (I974)

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Diagnosis requires:
Recurrent oral aphthous ulcers PLUS 2 of:
1. Aphthous genital ulceration
2. Uveitis
3. Cutaneous “pustular” vasculitis
4. Synovitis
5. Meningoencephalitis
Incomplete form: Recurrent oral aphthous ulcers PLUS > 1 of above criteria
Exclusions:herpes simplex infection, inflammatory bowel disease, Reiter’s syndrome and systemic lupus erythematosus (SLE)

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Behçet’s Disease
Research Committee of Japan Criteria (I972)

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Diagnosis requires: All 4-Major Criteria

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Major Criteria:

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1. Recurrent aphthous ulceration in the mouth
2. Skin lesions
• Erythema nodosum-ilke eruptions
• Subcutaneous thromhophlebitis
• Hyperirritability of the skin
3. Eye lesions
• Recurrent hypopyon iritis or iridocyclitis
• Chorioretinitis
4. Genital ulcerations
Incomplete type: 3/4 major criteria, or Recurrent hypopyon-iritis or typical chorioretinitis +1 other major criterion

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Minor Criteria:

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(Any number may present)
1. Arthritis symptoms (arthralgia, swelling, erythema)
2. Gastrointestinal lesions (e.g appen(licitis-like pains, melena)
3. Epididymitis
4. Vascular lesions (occlusion of blood vessels, aneurysms)
5. Central nervous system involvement
• Brain stem syndrome
• Meningo-encephalomyelitic syndrome
• Confusional type

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N.B.

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• Diagnosis of BD depends entirely on history taking and clinical Findings.
• Unlike the other two sets of criteria, The International Study Group criteria make 4/5 criteria mueoeutaneous.
• The sensitivity and specificity for the different diagnostic criteria:
International criteria (95%, 100%), 0 ‘Daffy criteria (100%, 89%), and Japanese criteria (100%, 95%)•
• Some clinical features appear to be more common in Japanese and Eastern Mediterranean patients as compared to American and European patients, and some have argued for the inclusion of ethnic origin to the international criteria.6
• Inclusion of recurrent oral ulceration as a non-optional criterion may exclude 3% of patients considered to have definite BD. Some have argued that in adult BD, recurrent oral ulcerations should not he a mandatory finding9
• There are no diagnostic laboratory tests specific for BD.
• Due to regional differences in positive rates, the pathergy test is no longer considered pathognomonic of BD.

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Birt-Hogg-Dube Syndrome

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In 1988, three Canadian physicians, Birt (Dermatologist), Hogg (Pathologist) and Dube (Internist), described a kindred with an autosomal dominant condition and multiple small grey to skin colored papules on the face, neck and upper trunk

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Diagnostic Criteria Birt Hogg Dube:

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Triad of:
1. Fibrofolliculomas
2. Trichodiscomas
3. Acrochordons

Other associations include:
1. Multiple bidradenomas
2. Renal cell carcinoma and renal oncocytoma
3. Parotid oncocytoma
4. Colonic polyps and adenocarcinoma
5. Parathyroid adenomas
6. Recurrent spontaneous pneumothorax
7. Flecked chorioretinopathy
8. Oral papular lesions
9. Multiple lipomas

Front 17

Carney Complex (CNC)

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The Carney complex includes LAMB (lentigines, atrial myxoma, mucocutaneous myxomas and blue nevi) and NAME (nevi, atrial myxomas, myxcoid neurofibromatosis, and ephilidcs and endocrine neoplasia). It can have autosomal dominant or X-Iinkcd inheritance.

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Stratakis, Kirschner & Carney Criteria (2001)
Diagnosis requires:

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>2 major criteria or 1 major criterion PLUS 1 minor criterion

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Major Criteria:

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1. Skin: spotty pigmentation with typical distribution (e.g. inner or outer canthi, conjunctiva, lips, vaginal and penile mucosa)
2. Myxoma: cutaneous and mucosal with histologic confirmation
3. Myxoma: cardiac with histologic confirmation
4. Breast myxomatosis with histologic confirmation or fat-suppressed MRI suggestive of this diagnosis
5. Primary pigmented nodular adrenocortical disease (PPNAD) with histologic confirmation, or paradoxical positive response of urinary glucocorticoids to dexamethasone administration during Liddle’s test
6. Acromegaly 2° to GH-producing adenoma with histologic confirmation
7. Large-cell calcifying Sertoli cell tumor (LCCSCT) with histologic confirmation or characteristic calcification on testicular ultrasound
8. Thyroid carcinoma with histologic confirmation, or multiple hypoechoic nodules on thyroid ultrasound in a young patient
9. Psammomatous melanotic schwannoma with histologic confirmation
10. Epithelioid blue nevus (multiple) with histologic confirmation
11. Breast: ductal adenoma (multiple) with histologic confirmation
12. Osteochondromyxoma with histologic confirmation

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Minor Criteria:

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1. Affected 1st degree relative
2. Inactivating mutation of the PRKAR1A gene

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Otherfindings suggestive, but not diagnostic, of CNC:

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1. Intense freckling: without darkly pigmented lesions or typical distribution
2. Blue nevus, usual type (if multiple)
3. Caf&au-lait mactiles (CALM) or other “birthmarks”
4. Multiple acrochordons (skin tags) and other skin lesions (e.g. lipomas)
5. Pilonidal sinus
6. IGF-I levels, abnormal oral glucose tolerance test or paradoxical GH responses to TRH testing in the absence of clinical acromegaly
7. Colonic polyps (often in association with acromegaly)
8. Hyperprolactinemia (often mild and usually associated with acromegaly)
9. Careliomyopathy
10. Single benign thyroid nodule in a young patient or multiple thyroid nodules in an older patient (as detected by ultrasound)
11. History of Cushing’s syndrome, acromegaly, or sudden death in extended family
12. Family history of carcinoma (esp. thyroid, colon, pancreas, ovary, and other benign or malignant tumors)

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Churg-Strauss Syndrome (CSS)

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American pathologists Jacob Churg and Lotte Strauss first described the condition in 1951 as a necrotizing, hypereosinopbilic vasculitis.

Front 23

ACR Criteria (1990)
Churg-Strauss Syndrome (CSS)

Diagnosis requires: >4 criteria

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1. Asthma
2. Eosinophilia> 10% on a differential WBC count
3. Mono- or poly-neuropathy: e.g. glove/stocking distribution; attributable to a systemic vasculitis
4. Pulmonary infiltrates (non-fixed): migratory or transitory pulmonary infiltrates on CXR
5. Paranasal sinus abnormality: Hx of paranasal sinus pain/tenderness or X-ray opacification of paranasal sinuses
6. Biopsy: artery, arteriole, or venule showing an accumulation of eosinophils in extravaseular areas
** History of allergy (non-drug): includes seasonal, food, contactants and others; presence of this feature improves the sensitivity of the criteria
N.B.2
Presence of 4 of 6 criteria: SN: 85%, SP: 99.7%
Skin manifestations are common in CSS and often the site of a positive biopsy. They include: palpable purpura (45%), maculopapular rash (40%) and subcutaneous nodules (20%).

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Cowden Syndrome (CS) (Multiple Hamartoma Syndrome)

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Cowden syndrome is an autosomal dominant condition characterized by the development of hamartomas in many organs including the skin, gastrointestinal tract, central nervous system, breast and thyroid.The eponym originates from the surname of the first patient reported, a 20- year-old woman with characteristic cutaneous, oral and acral papuics in addition to fibrocystic breast disease, breast cancer and multiple thyroid adenomas.

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International Cowden Consortium: Operational Criteria (Version 2000)

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Diagnosis requires:
1. Mucocutaneous lesions alone if:
a. > 6 Facial papules, of which >3 are trichilemmomas, or
b. Cutaneous facial papules arid oral mucosal papillomatosis, or
c. Oral mucosal papillomatosis and au-al keratoses, or
d. > 6 Palmoplantar keratoses
OR
2. 2 major criteria, where I includes macrocephaly or Lhermitte-Duclos disease (LDD)
OR
3. 1 major + 3 minor criteria
OR
4. 4 minor criteria

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Operational diagnosis in a family where one person is diagnostic for
CS requires:

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1. Pathognomonic criterion
2. Any 1 major criterion + / - minor criteria
3. 2 minor criteria

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Pathognomonic Criteria: (Mucocutaneous Lesions)

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1. Trichilemmomas: face
2. Keratoses (au-al)
3. Papillomatous papules
4. Mucosal lesions

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Major Criteria:

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1. Breast cancer
2. Thyroid cancer (non-medullary, especially follicular)
3. Macrocephaly (> 95th percentile)
4. Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum)
5. Endometrial cancer

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Minor Criteria:

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1. Other thyroid lesions (e.g. adenoma, multinodular goiter)
2. Mental retardation (IQ < 75)
3. GI hamartomatous polyposis
4-. Breast: fibrocystic disease
5. Lipomas
6. Fibromas
7. GU tumours (e.g. renal cell carcinoma, uterine fibroids) or malformation

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Dermatomyositis (DM)

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Two sets of criteria arc presented, starting with the most recent set.

Front 31

DERMATOMYOSITIS
Tanimoto et al Criteria (1995)

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Diagnosis requires:
> 5 criteria (>1 skin lesion PLUS > 4 other criteria)

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I. Skin lesions

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a. Heliotrope rash (violaceous edematous plaque on the upper palpebra)
b. Gottron’s sign/papules (violaceous keratotic maeules or papules on the extensor surface of finger joints)
c. Erythema on extensor surface of extremity joints! violaceous papules over elbows or knees

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Other Criteria

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2. Proximal muscle weakness (upper or lower extremity, and trunk)
3.  Serum creatinine kinase (CK) or aldolase level
4. Muscle pain on grasping or spontaneous pain
S. Myogenic changes on EMG (short-duration, polyphasic motor unit potentials with spontaneous fibrillation potentials)
6. Positive anti-Jo-l antibody
7. Nondestructive arthritis or arthralgias.
8. Systemic inflammatory signs (fever> 37° C in axilla,  serum CRP or  ESR> 20mm/h)
9. Pathological findings compatible with inflammatory myositis (inflammatory infiltration of skeletal muscle with degeneration or necrosis of muscle fibers; active phagocytosis, central nuclei, or evidence of active regeneration may be seen)
N.B.
. SN:94.l%,SP:90.3%.3

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DERMATOMYOSITIS
Bohan & Peter Criteria (1975)

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(original criteria)
Diagnosis requires:
Definite disease: 4 criteria (3 + cutaneous eruption)
Probable disease: 3 criteria (2 + cutaneous eruption)
Possible disease: 2 criteria (1 + cutaneous eruption)

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Criteria

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1. Symmetrical weakness of the limb girdle muscles and anterior neck flexors, progressing over weeks to months, +/- dysphagia or respiratory muscle involvement
2. Muscle biopsy evidence of necrosis of myofibers, phagocytosis,
regeneration with basophils, large vesicular sarcolemmal nuclei, and prominent nucleoli, atrophy in a perifascicular distribution, variation in fiber size and an inflammatory exudate (often perivascular)
3. T Serum skeletal-muscle enzymes, especially CK and often aldolase, AST/SGOT,AL.T/SGPT, and LDH
4. Electromyographic triad of short, small, polyphasic motor units, fibrillations, positive sharp waves and insertional irritability, and bizarre, high frequency repetitive discharges
5. Any one of the characteristic dermatologic features of the rash of DM

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Exclusion criteria:

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1. Central or peripheral neurologic 7. Infectious myopathy
disease 8. Malnutrition
2. Congenital muscuiar dystrophies 9. Metabolic or endocrine myopathies
3. Drugs or toxins associated with 10. Muscular dystrophy
myopathies 11. Myasthenia gravis
4. Giant cell arteritis and other forms 12. Rhabdomyolysis of known cause
of vascuiitis 13. Rubella vaccination
5. Granulomatous myositis 14. Sarcoidosis
6. Guillain-Barre syndrome
N.B.
• SN:93%,SP:93%.°
• In men stomach cancer and lymphoma, and in women ovarian cancer, are highly associated with DM relative to the normal population

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Dermatomyositis Sine Myositis
(Amyopathic dermatomyositis)

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Euwer & Sontheimer Criteria (1993)

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Diagnosis requires:

Back 38

1. Cutaneous changes pathognomonic of dermatomyositis
2. Skin biopsy fIndings compatible with dermatomyositis
3. No clinical evidence of proximal motor weakness withuni 2 years of skin disease
4. Normal skeletal enzyme levels for 2 years after appearance of skin lesions

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Drug Rash with Eosinophilia and Systemic Symptoms (DRESS)

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Bocquet, Bagot & Roujeau Criteria (1996)

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Diagnosis requires: All 3 criteria must be present

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1. Cutaneous drug eruption (usually widespread, morbilliform, erythrodermic or papulopustular + /- vesicles)
2. Hematologic abnormality: 1 or both of:
a. Eosinopbilia > l500/pL
b. Atypical lymphocytes
3. Systemic involvement: >1 of:
a. Lymphadenopathy > 2cm
b. Hepatitis (liver transaminases >2 times normal)
c. Interstitial nephritis
d. Interstitial pneumonitis
c. Carditis

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Implicated Drugs:

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• Aromatic anticonvulsants (e.g carbamazepinc, phenobarbital, phenytoin)
• Sulfonamides
• Less often: allopurinol, calcium-channel blockers, dapsone, minocycline, nevirapinc, ranitidine, sulfasalazine, thalidomide
N.B.
• DRESS is the proposed term to replace hypersensitivity syndrome.
• DRESS usually develops 2-6 weeks after a drug is first used (later than most idiosyncratic cutaneous reactions) or sooner if it is a rechallenge.

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Ehlers-Danlos Syndrome (EDS)

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The Ehiers-Danlos syndrome is a heterogeneous group of inherited disorders of connective tissue named after Danish dermatologist Edvard Ehlers and French dermatologist Henri- Alexander Danlos. Clinical features include joint hypermobility and hyperextensible, fragile skin. The six major types are listed below.

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Beighton et al Criteria (1997)

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for EDS ==> 6 types of note

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Classical Type (including Gravis and Mitis)

N.B.
• Autosomal dominant (AD) inheritance.
• Mutations in prooc 1(V) or prooc2(V) chains of typeV collagen (COL5A1, C0L5A2) in some families.

Back 44

Major Criteria:
i. Skin: hyperextensible
ii. Scars: widencd, atrophic
iii. Joints: hypermobile
Minor Criteria:
i. Skin: smooth, velvety
ii. Molluscoid pseudotumors (fleshy lesions associated with scars)
iii. Subcutaneous spheroids (small, hard, spherical, mobile subcutaneous bodies often on shins and forearms which may be calcified and detectable
radiologically)
iv. Joint hypermobility complications (e.g sprains, subluxations, pes planus)
v. Muscle hypotonia and delayed gross motor development
vi. Easy bruising
vii. Tissue extensibihty and fragility complications (e.g hiatal hernia, anal prolapse in childhood, cervical insufficiency)
viii. Surgical complications (e.g post-operative hernias)
ix. Positive family history
x. Prematurity of affected newborns

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Hypermobility Type

N.B.
• Autosomal dominant inheritance.

Back 45

Major Criteria:
i. Skin: hyperextensihle and/or smooth, velvety
ii. Joints: generalized hypermobility
Minor Criteria:
i. Joints: recurrent dislocations (esp. shoulder, patella,TMJ)
ii. Pain: chronic joints/limb (early in onset, can be debilitating)
iii. Positive family history

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Vascular Type

N.B.
• Autosomal dominant inheritance.
• Type III collagen gene (COL3AI) defect.

Back 46

* Presence of >= 2 Major Criteria is highly suggestive.

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Major Criteria:

Back 47

i. Skin: thin, translucent
ii. Arterial/intestinal/uterine fragility or rupture (mid-sized arteries most commonly involved; arterial rupture is #1 cause of sudden death)
iii. Bruising: extensive (must rule out child abuse and haematological disorders)
iv. Characteristic fades: decreased subcutaneous adipose tissue, especially of face and limbs

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Minor Criteria:

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i. Acrogeria
ii. Small joints: hypermobility (usually limited to digits)
iii. Tendon and muscle rupture
iv. Clubfoot
Varicose-veins: early onset
vi. Arteriovenous, carotid-cavernous sinus fistula
vii. Pneumothorax/pneumohemothorax
viii. Gingival recession
ix. Positive family history, sudden death in a close relative

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Kyphoscoliosis Type

N.B.
• Autosomal recessive.
• Caused by a deficiency of lysyl hydroxylase.

Back 49

* Presence of 3 Major Criteria in an infant is suggestive.

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Major Criteria:

Back 50

i. Joints: generalized laxity
ii. Muscle hypotonia: severe at birth; leads to delayed gross motor development
iii. Scoliosis: at birth, progressive
iv. Ocular: scleral fragility and rupture of ocular globe

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Minor Criteria:

Back 51

i. Tissue fragility, atrophic scars
ii. Bruising: easily
iii. Arterial rupture
iv. Marfanoid habitus
v. Microcornea
vi. Osteopenia: on radiographic imaging
vii. Positive family history (e.g. affected siblings)

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Arthrochalasia Type

N.B.
• Autosomal dominant (types A and B).
• Retention of amino-terminal propeptide of type I collagen.

Back 52

EDS

Front 53

Major Criteria:

Back 53

i. Joints: severe generalized hypermobility, with recurrent subluxations
ii. Hip dislocation: congenital, bilateral

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Minor Criteria:

Back 54

i. Skin: hyperextensible
ii. Tissue fragility with or without abnormal scarring
iii. Bruising: easily
iv. Muscle hypotonia
v. Kyphoscoliosis
vi. Osteopenia: mild on radiographic imaging

Front 55

Dermatosparaxis Type

N.B.
• Autosomal recessive inheritance.

Back 55

EDS

Front 56

Major Criteria:

Back 56

i. Skin: severe fragility (wound healing not impaired, scars arc not atrophic)
ii. Skin: redundant, sagging (may resemble cutis laxa)

Front 57

Minor Criteria:

Back 57

i. Skin: soft, doughy
ii. Bruising: easily
iii. Premature rupture of fetal membranes
iv. Large hernias (e.g. umbilical, inguinal)

Front 58

Gianotti-Crosti Syndrome (GCS)

Back 58

The syndrome was first described by Italian dermatologists Gianotti and Crosti as a symmetrical self-healing erythematous to flesh-colored papiilar eruption on the face, buttocks and extremities of children. It has been associated with a number of viral (hepatitis A, B, C, EB CMV, Coxsackie A16, B4- and B5, adenovirus, RSV, parainfluenza virus, parvovirus B19, rotavirus, rubella virus, HIV- 1), bacterial (group A 3-hemolytic streptococci, Mycobacterium avium-intracellularc complex) infections and immunizations (polio vaccine enterovirus, diphtheria immunization, pertussis immunization, influenza virus immunization)

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GIANOTTI-CROSTI SYNDROME

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Chuh Criteria (2001)

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Diagnosis requires: all 4 criteria below:

Back 60

I. Presence of all 4 positive clinical features on at least I occasion
2. Absence of all negative clinical features
3. GCS is the most likely diagnosis based on clinical judgment
4. If a lesional biopsy is performed, findings are consistent with GCS

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Positive Clinical Features:

Back 61

1. Monomorphous, flat-topped, pink-brown papules or papulovesicles 1-10mm in diameter
2. >= 3 sites involved: cheeks, buttocks, extensor surfaces of forearms, and extensor surfaces of legs
3. Symmetry
4. Duration of 10 days or more

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Negative Clinical Features:

Back 62

1. Extensive truncal lesions
2. Scaly lesions

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N.B.

Back 63

• Presence of all of the positive clinical features is 100% sensitive for diagnosis of GCS.
• Rash duration of 10 days is most specific (61.3%) and most predictive (47.8%) for GCS.
• Symmetry is least specific (19.4%) and least predictive (30.6%) for GCS.
• The Koebner phenomenon is commonly seen.
• GCS lesions are non-pruritic, blanchable, but may be hemorrhagic or petechial.
• Lymphadenopathy and hepatosplenomegaly were the classic signs for the old hepatitis-related diagnosis of GCS, but their absence does not exclude the
diagnosis of GCS.

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Henoch-Schönlein Purpura (HSP)

Back 64

Henoch-Schönlein purpura was named after German physicians Johann Schönlein, who described cases of purpura associated with arthritis, and Eduard Henoch, who added the gastrointestinal and renal manifestations to that description.

Front 65

HSP: Helander, De Castro & Gibson Criteria (1995)

Back 65

Diagnosis requires: >= 3 of the criteria below

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N.B.’
SN&SP>90%.

Back 66

1. Direct immunofluorescence (DIP) suggesting vasculitis, with presence of IgA deposits
2. Age <= 20 at onset
3. GI involvement
4. Upper respiratory tract infection prodrome
5. Renal Biopsy: Mesangioproliferative GN + /- IgA deposition

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HSP: ACR Criteria (1990)

Back 67

Diagnosis requires: >= 2 of 4 criteria below
1. Palpable purpura: raised hemorrhagic lesions not due to thrombocytopenia
2. Age <= 20 at condition onset
3. Bowel angina: diffuse abdominal pain often worse after meals, or bowel ischemia with related hematochezia
4. Vessel wall granulocytes on biopsy: histology shows granulocytes in arteriole or venule walls

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N.B.

Back 68

• Presence of >= 2 criteria: SN: 87.1%, SP: 87.7%.
• The first 2 criteria appear to be the best discriminators for HSP.
• The original tetrad of HSP was: palpable purpura, arthritis, GI involvement, and nephritis.
• HSP is the most common systemic vasculitis in children.

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HSP: Michel et al Criteria (I992)

Back 69

(Used For dffrrentiating hypersensitivity vasculitis (HV)from HSP)

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Diagnosis requires: >= 3 criteria below

Back 70

1. Palpable purpura
2. Bowel angina
3. GI bleeding
4. Hematuria
5. Age < 20 at onset
6. Not caused by medications

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N.B.

Back 71

Presence of >= 3 criteria correctly classified 87.1 o,/ of patients with HS while presence of < 3 criteria classified 74.2% of patients with HV

Front 72

Hereditary Hemorrhagic Telangiectasia (HHT)
(Rendu-Osler-Weber Syndrome)

Back 72

This autosomal dominant condition was originally described by Flenri Rendu (1896), SirWillias Osler (1901) and Frederick Weber (1907).

Front 73

Curaçao Criteria (2000)

Back 73

Diagnosis requires:
Definite 3 criteria
Possible or suspected 2 criteria
Unlikely <2 criteria

Front 74

Criteria:

Back 74

1. Epistaxis (spontaneous, recurrent nose bleeds)
2. Telangiectases (multiple, and at characteristic sites: lips, oral cavity, fingers, nose)
3. Visceral lesions (e.g. GI telangiectasia +/- bleeding, pulmonary AVM, hepath AVM, cerebral AVMs, spinal AVM)
4. Family history (first degree relative with HHT using these criteria)

Front 75

N.B.

Back 75

• HHT may not manifest until late in life.
• Coagulation disorders should be excluded.
• Visceral abnormalities in children as noted above should prompt careful evaluation of family members.

Front 76

Hypersensitivity Vasculitis (HSV)

Back 76

HYPERSENSITIVITY VASCULITIS
ACR Criteria (1990)

Front 77

Diagnosis requires: >= 3 of 5 criteria below

Back 77

1. Age> 16 years at disease onset
2. Medication taken at disease/symptoms onset
3. Palpable purpura: not related to thromboc)rtopenia, doesn’t blanch
4. Maculopapular rash
5. Biopsy including arteriole and venule: histology showing granulocytes in a perivascular or extravascular location

Front 78

N.B.

Back 78

• Presence of >=3/5 criteria: SN: 71%, SP: 83.9%.
• Presence of eosinopbils in a venule or arteriole at any location on biopsy improves sensitivity.
• The most difilcult condition to differentiate from HSV is Henoch-Schönlein purpura (HSP); the main points of differentiation are the younger age group affected by HSP and preferred involvement of GI tract and kidneys by HSP (see Michel criteria under Henoch-Shönlein purpura on page 28).

Front 79

Kawasaki Disease/Syndrome
(Mucocutaneous Lymph Node Syndrome)

Back 79

Kawasaki disease was first described by Japanese pediatrician Tomisaku Kawasaki in 1967 as “acute febrile mucocutaneous syndrome with lymph node involvement.” Young children and infants are typically affected.

Front 80

Morens & O’Brien Criteria (1978)

Back 80

Diagnosis requires:
Fever lasting >= 5 days PLUS 4/5 of the following criteria PLUS
Other illnesses with similar clinical signs must be excluded.

Front 81

Criteria:

Back 81

1. Polymorphous rash/exanthem, but non-vesicular
2. Bilateral conjunctival injection
3. 1 or more of the following mucous membrane changes:
i. Diffuse injection of oral & pharyngeal mucosa
ii. Erythema or fissuring of lips
iii. Strawberry tongue
4. Acute, non—purulent cervical lymphadenopathy (at least 1 cervical lymph node> 1.5cm)
5. >=1 of the following extremity changes:
i. Erythema of palms and/or soles
ii. Indurated edema of hands and/or feet
iii. Desquamation of distal extremities

Front 82

N.B.

Back 82

1. t ESR, anemia, and thrombocytosis support the diagnosis of KD.
2. The cause is still unknown, and there is no specific diagnostic test.
3. Patients with fever and <4 of the principal criteria can be diagnosed with KD if coronary artery abnormalities including aneurysm formation develop.

Front 83

Lyme Disease

Back 83

L.ymc disease is caused by the Ixodes tick-borne spirochete Borrelia burgdorferi, and named after Lyme, Connecticut, where an unexpected number of residents were found to have a new and unusual illncss.The tick bite is usually painless and not noticed. During the first stage, erythema chronicum migrans (ECM) develops 3-30 days after a tick bite at the site of the bite (occurs in 60- 80% of pta.), then fades in 3-4 weeks.
The spirochetes disseminate widely within days to weeks and multi-organ involvement may occur. In the skin, secondary ECM, urtiearia, crythema nodosum and ly-mphocytoma cutis may occur. Other features include large joint oligoarthritis, meningitis, encephalitis, Bell’s palsy, cranial /radicular neuritis, lymphadenopathy, sp1enoinegal myopericarditis, atrioventricular block, conjunctivitis, keratitis, optic neuropathy, malaise, fatigue, cough, sore throat and microscopic hematuria.
Weeks to years after the bite, the third stage is seen with acrodermatitis chronis atrophicans, scicrodermoid lesions, arthritis, encephalomyclitis, paraparesis, keratitis and fatigue.

Front 84

CDC Criteria (1991)

Back 84

Diagnosis requires:

Front 85

1. a. Erythema migrans (EM) > 5cm diagnosed by a physician or
h I Late manifestation:
i. Recurrent brief swollen joints
ii. Acute onset of secondary or tertiary cardiac conduction defect
iii.Meningitis, cranial neuropathy, radiculoneuritis, encephalomyeitis with
intrathecal antibodies produced

Back 85

2. Laboratory confirmation of Borrelia burgdoferi infection:
a. Culture
b. Antibodies: CSF or Serum (1gM peaks at 3-6 weeks, then IgG 1 and IgG3
develop)
c. Acute and convalescent sera documenting rising titers
h. A two-test approach using a sensitive enzyme immunoassay or
immunofluorescence antibody followed by Western blot is
recommended

Front 86

N.B.

Back 86

Smouha et al stated that Lyme disease should be suspected as a cause of facial nerve palsy even in the absence of other clinical symptoms in patients living in or visiting endemic areas

Front 87

Marfan Syndrome

Back 87

This autosomal dominant syndrome is named after French pediatrician Antoine Marfan, a professor of paediatrics who presented a 5-year-old child with skeletal abnormalffles in Paris in
1896

Front 88

De Paepe et al Criteria (1996)’
(modified from the 1986 Berlin Nosology)

Back 88

Diagnosis requires

Front 89

For the index case:

Back 89

• If the family/genetic history is not contributory, major criteria in at least 2 different organ systems and involvement of a third organ system
• If a mutation known to cause Marfan syndrome in others is detected, 1 major criterion in an organ system and involvement of a second organ system

Front 90

For a relative of an index case:

Back 90

• Presence of a major criterion in the family history and 1 major criterion in an organ system and involvement of a second organ system

Front 91

Skeletal System

Back 91

For the skeletal system to be considered involved, >= 2 of the major criteria or 1 major + 2
minor criteria must be present.

Front 92

Major Criteria:

Back 92

1. Pectus carinatum
2. Pectus excavatum requiring surgery
3. Reduced upper to lower segment ratio or arm span to height ratio greater than 1.05
4. Wrist and thumb signs
5. Scoliosis of> 200 or spondylolisthesis
6. Reduced extension at the elbows (< 170°)
7. Medial displacement of the medial malleolus causing pes planus
8. Protrusio acetabulae of any degree (on radiographs)

Front 93

Minor Criteria:

Back 93

1. Pectus excavatum of moderate severity
2. Joint hypermobility
3. Highly arched palate with crowding of teeth
4. Facial appearance (dolichocephaly, malar hypoplasia, enophthalmos, retrognathia, down-slanting palpebral fissures)

Front 94

Cardiovascular System

Back 94

For the cardiovascular system to be involved, 1 major criterion or only I of the minor
criteria must be present.

Front 95

Major Criteria:

Back 95

1. Dilatation of the ascending aorta with or without aortic regurgitation and involving at least the sinuses of Valsalva
2. Dissection of the ascending aorta

Front 96

Minor Criteria:

Back 96

1. Mitral valve prolapse with or without mitral valve regurgitation
2. Dilatation of the main pulmonary artery, in the absence of valvular or peripheral pulmonic stenosis or any other obvious cause, below the age of 40 years
3. Calcification of the mitral arinulus below the age of 40 years
4. Dilatation or dissection of the descending thoracic or abdominal aorta below the age of 50 years

Front 97

Ocular System

Back 97

For the ocular system to be involved, >= 2 of the minor criteria must be present.

Front 98

Major Criterion:

Back 98

1. Ectopia lens

Front 99

Minor Criteria:

Back 99

1. Abnormally flat cornea (measured by keratometry)
2. Increased axial length of globe (measured by ultrasound)
3. Hypoplastic iris or hypoplastic ciliary muscle causing decreased miosis

Front 100

Pulmonary System

Back 100

For the pulmonary system to be involved, I of the minor criteria must be present.

Front 101

Major Criteria

Back 101

: none

Front 102

Minor Criteria:

Back 102

1. Spontaneous pneumothorax or
2. Apical blebs (on CXR)

Front 103

Skin & Integument

Back 103

For the skin and integument to be involved, 1 of the minor criteria must be present.

Front 104

Major Criteria

Back 104

: none

Front 105

Minor Criteria:

Back 105

1. Stretch marb not associated with marked weight changes, pregnancy or repetitive stress
2. Recurrent or incisional hernia

Front 106

Dura

Back 106

For the dura to be involved, the major criterion must be present.

Front 107

Major Criterion:

Back 107

Lumbosacral dural ectasia by CT or MRI

Front 108

Minor Criteria

Back 108

: none

Front 109

Family/Genetic History

Back 109

For thefamily/genetic history to be contributory, 1 oF the major criteria must be present.

Front 110

Major Criteria:

Back 110

• Having a parent, child or sibling who meets these diagnostic criteria independently
• Presence of a mutation in FBN I known to cause the Marfan syndrome
• Presence of a haplotype around FBN 1, inherited by descent, known to be associated with unequivocally diagnosed Marfan syndrome in the family

Front 111

Minor Criteria

Back 111

: none

Front 112

Muir-Torre Syndrome (MTS)

Back 112

In 1967, Sir Edward Muir (British surgeon), Bell and Barlow,’ and in 1968, America, dermatologist DouglasTorre2 described this autosomal dominant genodermatosis characterize by sebaceous tumors, keratoacanthomas and internal malignancy.

Front 113

MUIR-TORRE SYNDROME
Cohen et al Criteria (I995) N.B.
The most common internal malignancies are gastrointestinal, followed by genitourinary.

Back 113

Diagnosis requires:
1. At least one sebaceous tumor (sebaceous adenoma, sebaceous epitheioma, or sebaceous carcinoma) or keratoacanthoma with sebaceous differentiation and a visceral malignancy, in the absence of any known predisposing factors

Front 114

OR

Back 114

2. In the absence of sebaceous tumor,
i. Multiple keratoacanthomas
ii. Multiple visceral malignancies
iii. Family history of MTS

Front 115

Neurofibromatosis

Back 115

Neurofibromatosis 1 and II are genetic diseases with autosomal dominant inheritance anda high rate of new mutations. NFl is named after German histologist and pathologist Fricdrich von Rccklinghausen

Front 116

Neurofibromatosis I
(von Recklinghausen’s/Oassic neurofibromatosis/Peripheral neurofibromatosis)
NIH Criteria (1987)’

Back 116

Diagnosis requires:
>= 2 cardinal clinical features

Front 117

Cardinal clinicalfeatures:

• These criteria refer to Caucasians. Unfortunately, little work has been done for minimal criteria in non-Caucasian persons.

Back 117

1. >= 6 café-au-lait macules> 5mm ni greatest diameter in pre-pubertal individuals and> 15mm in greatest diameter in post-pubertal individuals
2. >= 2 neurofibromas of any type or 1 plexiform neurofibroma
3. Freckling in the axillary or inguinal regions (Crowe’s sign)
4. Optic nerve or pathway glioma
5. >= 2 Lisch nodules (iris hamartomas)
6. A distinctive bony lesion (e.g. sphenoid dysplasia or thinning of the long bone cortex, with or without pseudarthrosis)
7. A 1ST degree relative (parent, sibling, or offspring) with NF I by the above criteria

Front 118

N.B.

Back 118

• 97% of NF- 1 patients meet the above criteria by age 8; all do so by age 20.
• For children < 8 years of age, these criteria are less helpful. For children < 1 year of age, only 30% of patients have 1 of the cardinal clinical features.
• The onset of the clinical features:
o Café au-lait mactiles (CALM): usually present by age 1; unlikely to develop after age 4.
o Axillary freckling: usually develops by age 7.
o Lisch nodules: usually develop before age 10.
o Neurofibroma: usually present by age 20.
o Symptomatic optic glioma usually diagnosed by age 3.
o Characteristic osseous lesions present usually by age 1.
• It has been recommended that young children who have >= 6 caf&au-lait mactiles > 5mm in largest diameter, but lacking in other cardinal features be followed as if they have NFl. Most will develop other features with time.
• For those < 8 years-old in whom the diagnosis is in question, other features such as short stature, macrocephaly, and unidentffied bright objects on head MRI may aid in the diagnosis, as might routine radiographic screening for pseudarthrosis, sphenoid wing dysplasia, and dysplastic vertebrae.

Front 119

Neurofibromatosis II

(Biateral acoustic neurofibromatosis /Central neurofibromatosis)

Back 119

Two sets of criteria for Neurofibromatosis II are given below, starting with the most recent one.

Front 120

NF II National Neurofibromatosis Foundation (NNFF) Criteria (l997)

Back 120

Diagnosis requires:

Front 121

Confirmed or Definite NF-2

Back 121

1. Bilateral vestibular schwannomas
OR
2. 1ST -degree family relative with NF-2 PLUS
a. unilateral vestibular schwannoma < age 30 years or
b. any 2 of:
i. Meningioma
ii. Schwannoma
hi. Glioma
iv. Juvenile lens opacity (e.g. posterior subcapsular or cortical cataract)

Front 122

Presumptive or Probable NF-2

Back 122

1. Unilateral vestibular schwannoma < age 30 years PLUS >= 1 of:
i. Meningioma
ii. Schwannoma
iii. Glioma
iv. Juvenile lens opacity (e.g posterior subcapsular or cortical cataract)
2. >= 2 meningiomas PLUS
a. unilateral vestibular schwannoma < age 30 years or
b. >= l of:
i. Schwarinoma
ii. Glioma
iii. Juvenile lens opacity (e.g. posterior subcapsular or cortical cataract)

Front 123

NF II  NIH Criteria (1987)

Back 123

Diagnosis requires:
1. Bilateral 8th nerve masses seen on CT or MRI
OR
2. 1ST -degree family relative with NF-2 and either
i. unilateral 8th nerve mass or
ii. >= 2 of:
a. Neurofibroma
b. Meningioma
c. Sehwannoma
d. Glioma
e. Juvenile posterior subcapsular lenticular opacities

Front 124

N.B.

Back 124

• The specificity for each set of criteria is approximately 99%.
• Bilateral vestibular schwannomas are pathognomonic for NF-2, but they are not always present (41% not present in one study of NF- 2 patients)
• Only the NNFF criteria are able to make the diagnosis of NF-2 in people who do not have bilateral vestibular schwannomas or a family history of NF- 2, but who do have other characteristic features.
• Adding mononeuropathy (e.g foot drop, wrist drop, facial nerve palsy, or third nerve palsy) and/or generahzed peripheral neuropathy as diagnostic criteria would improve sensitivity, but has yet to be included in formal criteria. Also, genetic testing will likely be included in future criteria.

Front 125

Papular-Purpuric Gloves and Socks Syndrome

Back 125

In 1990, the syndrome was first described in 5 patients aged 16-31 years by Harms, Feldmann and Saurat.’ The syndrome has been associated with certain viruses, particularly parvovirus
B19.

Front 126

Papular-Purpuric Gloves and Socks Syndrome

Halasz, Cormier & Den Criteria (1990)

Back 126

Diagnosis requires:
1. Fever
2. Pruritic or painful edema arid erythema followed by petechiae on the hands and feet with abrupt demarcation at wrists and ankles
3. Oral involvement with petechiae and/or oral erosions
N.B.
Other features that may be seen include petechiae on the inner thighs, inguinal area, buttocks, knees and elbows, lymphadenopathy and leukopenia. The condition usually resolves with desquamation in 1-2 weeks.

Front 127

Paraneoplastic Pemphigus

Back 127

Two different sets of criteria are presented.

Front 128

PARANEOPLASTIC PEMPHIGUS

Anhalt Criteria (1997)

Back 128

Diagnosis requires:
1. Painful mucosal erosions and polymorphous skin lesions in the context of an underlying neoplasm
2. Histopathology: vacuolar interface changes, keratinocyte necrosis, and intraepidermal acaritholysis
3. Direct immunofluorescence: intercellular epidermal IgG and C3 and often linear or granular C3 at the dermal-epidermal junction
4. Indirect immunofluorescence: serum autoantibodies that bind to the cell surface of monkey esophagus and also to urinary bladder epithelium
5. Serum autoantibodies against a complex of high-molecular weight proteins from keratinocyte extracts with molecular weights of 250, 230, 210, 190 and l7Okd

Front 129

N.B.

Back 129

• Immunoprecipitation is highly sensitive and specific, but not readily available.
• Immunoblotting is easier than immunoprecipitation, but not as sensitive.
• The criteria with high sensitivity (82-86%) and high specificity (83%- l00%) were:
i. Association with lymphoproliferative disorders
ii. Indirect immunofluorescence: presence of autoantibodies that labeled rat bladder
iii. Immunoblotting detection of autoantibodies against periplakin (190 kd) and envoplakin (210 kd).
• The criteria that were specific (8 3%- 100%), but had poor sensitivity (2 7%- 59%) were:
i. Association of oral erosions with bullous pemphigoid-like, erythema multiforme-like or lichen planus-like lesions
ii. Histopathology: suprabasal acantholysis with keratinocyte necrosis or vacuolar interface dermatitis or lichenoid infiltrate
iii. Presence of circulation or in vivo bound anti-epitheial cell surface and anti-basement membrane zone antibodies
iv. Immunoblot recognition of desmoplakin 1(250 kd) or BPAG 1 (230 kd).

Front 130

PARANEOPLASTIC PEMPHIGUS

Camisa & Helm Paraneoplastic Pemphigus Criteria (1993)

Back 130

Diagnosis requires: All 3 Major criteria or 2 Major PLUS >=2 Minor criteria

Front 131

Major Criteria:

Back 131

1. Polymorphous mucocutaneous eruption
2. Concurrent internal neoplasia
3. Characteristic serum immunoprecipitation findings

Front 132

Minor Criteria:

Back 132

1. Indirect immunofluorescence: positive cytoplasmic staining to rat bladder epithelium
2. Direct immunofluorescence of perilesional skin: Intercellular and basement membrane zone immunoreactants
3. Histopathology: acantholysis in biopsy specimen from >=1 anatomic site of involvement

Front 133

Pityriasis Rosea (PR)

Back 133

Chuh Criteria (2003)

Front 134

Diagnosis requires:

Back 134

All 3 major eriteria PLUS >= 1 minor criterion PLUS none of the exclusional criteria

Front 135

Major Criteria:

Back 135

1. Discrete oval or circular lesions
2. Scaling on most lesions
3. >= 2 lesions with peripheral collarette of scale and central clearing

Front 136

Minor Criteria:

Back 136

1. Distribution on trunk and proximal limbs with < 10% of lesions distal to mid-thigh and mid-upper-arm
2. Most lesions distributed along the ribs
3. Herald patch (not necessarily the largest) appearing >= 2 days before the generalized eruption

Front 137

Exciusional Criteria:

Back 137

1. Multiple small vesicles at the center of >=2 lesions
2. Most lesions on palms or soles
3. Clinical or serological evidence of secondary syphihs (e.g generalized lymphadenopathy)
• >= 2 lesions with peripheral collarette of scale and central clearing:
SN: 100%, SP: 81%.
• Distribution along ribs: SN: 77.8%, SP: 98.3%.
• Herald patch: SN: 27.8%, SP: 96.6%.
• Discrete oval or circular lesions, sealing on most lesions & distribution on trunk and proximal limbs: sensitive and non-specific.

Front 138

Polyarteritis Nodosa (PAN)

Back 138

POLYARTERITIS NODOSA
ACR Criteria (1990)

Front 139

Diagnosis requires: >= 3 criteria

Back 139

1. Weight loss >= 4kg due to illness
2. Livedo reticularis
3. Testicular pain/tenderness: not related to infection or trauma
4. Myalgias (diffuse), weakness, or leg tenderness
5. Mono- or poly-neuropathy
6. Diastolic BP > 90mm Hg
7. t Cr or BUN: t Cr> 1 .5mg/dl (> 132 pmol/L) or t BUN > 40mg/dl (>14 mmol/L): not due to dehydration or obstruction
8. Hepatitis B Virus (Ag orAb in serum)
9. Arteriographic abnormality: aneurysms or occlusions of visceral arteries
10. Biopsy of small or medium-sized artery containing PMN: presence of granulocytes and/or mononuclear leukocytes in artery wall

Front 140

N.B.

Back 140

SN: 82.2%, SP: 86.6%.

Front 141

Polyarteritis Nodosa, Cutaneous

Back 141

This is a localized form of PAN.

Front 142

CUTANEOUS POLYARTERITIS NODOSA
Bauzá, España & Idoate Criteria (2002)

Back 142

Diagnosis requires:
1. Presence of typical cutaneous lesions (nodules, livedo reticularis, ulceration, purpura)
2. Leukocytoclastic vasculitis in the arteries of the deep dermis or hypodermis
+ / - fibrinoid necrosis
3. Absence of visceral involvement at the time of diagnosis

Front 143

Pseudoxanthoma Elasticum (PXE)

Back 143

Pseudoxanthoma elasticuns is an inherited defect of elsstic tissue attributed to mutations in the transmemhrane adenosine triphosphate-hinding cassette transporter on chromosome 16

Front 144

PXE
Lebwohl et al Criterb (1994)

Back 144

Diagnosis requires:
Category I
• All 3 major criteria
Category IIa
• Angioid streaks
• Both minor criteria
Category lIb
• Angioid streaks
• Elastic fiber calcffication in non- lesional skin
Category lIc
• Angioid streaks
• Family history of PXE in first-degree relatives
Category lId
• Both minor criteria

Front 145

Major Criteria:

Back 145

1. Skin involvement: yellow cobblestone lesions in flexural locations
2. Skin histology of lesional skin: fragmentation of elastic tissue and calcffication (elastic tissue, calcium or von Kossa stains)
3. Ocular disease (angioid streaks, “peau d’ orange” appearance of flindus, or maculopathy) in adults> 20 years of age

Front 146

Minor Criteria:

Back 146

1. Histologic features of nonlesional skin: elastic fiber calcffication (elastic tissue and calcium or von Kossa stains)
2. Family history of PXE in first-degree relatives

Front 147

Reiter’s Syndrome/Reactive Arthritis

Back 147

Reiter’s syndrome/reactive arthritis was first described by Stoll in 1776 and Brodie in 1818, then German bacteriologist Hans Reiter in 1916 as a triad of arthritis, cnnjunctivitis and urethritis, following enteric (usually in cbildren and older adults) or venereal (usually young, sexually active people with multiple sexual partners) infections.
In the venereal form (endemic form), urethritis is usually the first symptom to develop, followed in a few days by conjunctivitis, then polyarticular asymmetrical artbritis. In the dysenteric form (epidemic form), the clinical triad usually onsets at the same time within 10- 50 days.

Front 148

REITER'S SYNDROME
Calm Criteria (1989)

Back 148

Diagnosis requires:
Seronegative asymmetric arthritis PLUS >= 1 other criterion

Front 149

Other criteria:

Back 149

1 Urethritis/cervicitis
2. Dysentery
3. Inflammatory eye disease
4. Mucocutaneous disease: e.g circinate balanitis, keratoderma blennorrhagica, nail changes (onycholysis, yellow discoloration, subungual hyperkeratosis) painless lingual or palatal oral ulcers, cervicitis, and sterile urethritis

Front 150

Exclusions:

Back 150

1. Ankylosing spondylitis
2. Psoriatic arthritis
3. Other rheumatic diseases

Front 151

Relapsing Polychondritis (RP)

Back 151

Two sets of diagnostic criteria are presented.The McAdarn criteria are presented first since the Damiani criteria depend on them.

Front 152

RELAPSING POLYCHONDRITIS

McAdam et al Criteria (1976)

Back 152

Diagnosis requires: >=3 criteria; histologic confirmation is helpful, but not
necessary

Front 153

Criteria:

Back 153

1. Auricular chondritis: bilateral recurrent
2. Arthritis: non-erosive, seronegative, inflammatory polyarthritis
3. Nasal chondritis
4. Ocular inflammation: e.g. conjunctivitis, keratitis, scleritis, or uveitis
5. Respiratory tract chondritis: e.g. tracheal or laryngeal cartilage
6. Audiovestibular damage: e.g. neurosensory hearing loss, tinnitus, or vertigo

Front 154

RELAPSING POLYCHONDRITIS

Damiani & Levine Criteria (1979)

Back 154

N.B.
• Laboratory and radiographic imaging are used to rule out other conditions rather than showing abnormalities specific to RP.
• Auricular chondritis is the most common clirdeal feature (88.6-90%), followed by artbropathy, nasal ehondritis, and ocular inflammation.

Front 155

Diagnosis requires:

Back 155

1. >=3 of McAdam criteria. Histologic confirmation not necessary
OR
2. >= 1 of MeAdam criteria + histologic confirmation
OR
3. Involvement of >= 2 separate anatomic locations, showing response to steroids and/or Dapsone.

Front 156

Rheumatic Fever, Acute

Back 156

Revised Jones Criteria (1997)

Front 157

Diagnosis requires:

(If however, chorea and carditis are present then demonstration of antecedent strep infection is not required to make the diagnosis)

Back 157

1. Supportive evidence of antecedent group A streptococcal infection (positive throat culture or rapid streptococcal antigen test, or elevated or rising streptococcal antibody titer) PLUS 2 major criteria
OR
2. 1 major PLUS 2 minor criteria
Major criteria
1. Carditis
2. Polyarthritis
3. Chorea
4. Erythema marginatum
5. Subcutaneous nodules
Minor criteria
Clinical findings:
i. Fever
ii. Arthralgia
Laboratory findings:
i. t Acute phase reactants (ESR, C-reactive protein)
ii. Prolonged PR interval
Reference

Front 158

Scleroderma, Systemic
(Diffuse systemic sclerosis)

Back 158

ARA Criteria (1980)

Front 159

Diagnosis requires:

Back 159

1 Major criterion or >= 2 Minor criteria

Front 160

Major criterion:

Back 160

1. Proximal seleroderma

Front 161

Minor Criteria:

Back 161

1. Sclerodactyly
2. Digital pitting scars of fIngertips or loss of substance of distal finger pad
3. Bilateral basilar pulmonary fibrosis

Front 162

N.B.

Back 162

SN:97%,SP:98%.

Front 163

Scleroderma, CREST Syndrome
(Limited systemic sclerosis)

Back 163

The mnemonic stands for:
Calciriosis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Front 164

Sezary Syndrome

Back 164

In 1938, French dermatologist Albert Sézary described the triad of erythroderma, enlarged peripheral lymph nodes and “monster” cells in the blood.’

Front 165

SEZARY SYNDROME
Russell-jones & Whittaker Criteria (2000)

Back 165

Diagnosis requires:
All of the following:
1) Erythroderma
2) Compatible skin histology
3) > 5% circulating atypical mononuclear ceUs
4) Peripheral blood T-cell clone, found by at least 1 of the following tests:
a. Presence of very large Sézary cells (>1 4$!)
b. Cytogenetic evidence of an abnormal clone
c. Loss of panT-cell antigens by immunophenotyping
d. T-cell clone by Southern blot or PCR single strand conformational polymorphism (SSCP) analysis

Front 166

Sjogren’s Syndrome

Back 166

In 1933, Swedish ophthalmologist Henrik Sjögren reported the association of xerophthalmia and xerostomia, with polyarthritis.

Front 167

San Diego Criteria (1986)

Back 167

Diagnosis requires:
Definite SS: Objective evidence of dryness of eyes/mouth and autoimmunity
including a characteristic minor salivary gland biopsy (criteria IA, IB, & IC)
Probable SS: Demonstration of reduced salivary function (criteria IA, IB- 1, & IC

Front 168

I. Primary Sjogren’s Syndrome

Back 168

A. Symptoms and objective signs of ocular dryness
1. Schirmer’s test: <8mm wetting/5 minutes, and
2 Positive Rose Bengal staining of cornea or conjunctiva to demonstrate keratoconjunctivitis sicea
B. Symptoms and objective signs of dry mouth
1. Decreased parotid flow rate using Lashley cups or other methods, and
2. Abnormal findings from biopsy of minor salivary gland (focus score of >= 2 based on average of 4 evaluated lobules)
C. Serologic evidence of a systemic autoimmunity
1. Elevated rheumatoid factor (RF) >= 1:320 or
2. Elevated antinuclear antibody (ANA) >= 1:320 or
3. Presence of anti-SS-A (Ro) or anti-SS-B (La) antibodies

Front 169

II. Secondary Sjogren’s Syndrome

Back 169

Characteristic signs and symptoms of SS (see above) + clinical features sufficient to allow a diagnosis of rheumatoid arthritis (RA), systemic lupus erytheinatosus (SLE), polymyositis, scleroderma, or hiliary cirrhosis.

Front 170

III. Exclusions

Back 170

1. Sarcoidosis 5. Primary fibromyalgia
2. Pre-existent lymphoma 6. Other known causes of autonomic neuropathy
3. HIV 7. Keratitis sicca
4. Hepatitis B or C 8. Salivary gland enlargement

Front 171

Still’s Disease, Adult

Back 171

George Fredriek Still, a British paediatrieian, deseribed a group of ehildren with inflammatory arthritis, fever, lymphadenopathy, splenomegaly and pleuroperiearditis in 1897. Although “Still’s disease” was first described in children and classifIed as a systemic onset juvenile chrome arthritis, it may onset de nova in adolescents and adults.

Front 172

STILL'S DISEASE
Cush et al Criteria (1989)

Back 172

Diagnosis requires:

Front 173

All of thefollowing:

Back 173

1. Fever >=39°C(102.2°F)
2. Arthralgia and/or arthritis
3. Rheumatoid factor (RE) <1:80
4. Antinuclear antibody (ANA) < 1:100

Front 174

PLUS
Any 2 of the following:

Back 174

1. WBC >= 15,000 cells/mm3
2. Still’s rash (an evanescent maculopapular eruption)
3. Pleuritis or pericarditis
4. Hepatomegaly or splenomegaly or generalized lymphadenopathy

Front 175

STILL'S DISEASE
Yamaguchi et al Criteria (I992)
N.B.
SN: 96. 2%, SP: 92. 1%.

Back 175

Diagnosis requires: >= 5 criteria including >=2 major criteria PLUS exclusion of disease listed in Exclusions

Front 176

Major Criteria:

Back 176

1. Fever 39°C (102.2°F) lasting >= 1 week
2. Artbralgia >= 2 weeks
3. Typical macular or maculopapular non-pruritic salmon-pink colored eruption usually appearing during fever
4. Leukocytosis ( 10,000/mm3) with >=80% granulocytes

Front 177

Minor Criteria:

Back 177

1. Sore Throat
2. Lymphadenopathy (recent & significant lymph node swelling) and/or splenomegaly (by palpation or ultrasound)
3. Liver dysfunction (t ALT, AST, and/or LDH) due to disease, and not drug allergy/toxicity or other cause)
4. Negative RF and ANA

Front 178

Exclusions:

Back 178

1. Infections (especially sepsis & mononucleosis)
2. Malignancies
3. Rheumatic diseases (especially PAN and rheumatoid vasculitis with extraarticular features)

Front 179

Streptococcal Toxic Shock Syndrome

Back 179

Working Group on Severe Streptococcal Infections Criteria (1993)

Front 180

Diagnosis reguires:

Back 180

Definite: Criteria Ia, 2 & 3
Probable: Criteria 1 b, 2 & 3 & other etiologies have been ruled out

Front 181

Criteria:

Back 181

1. Isolation of group A Streptococci (Streptococcus pyogenes)
a. From a normally sterile site (e.g. cerebrospirial, pleural, or peritoneal fluid, blood, tissue biopsy, etc)
b. From a normally non-sterile site (e.g. sputum, throat, vagina, superficial skin lesion, etc.)
2. The presence of hypotension (systolic blood pressure <= 90 mm Hg in adults or <5th percentile for children) or shock

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PLUS

Back 182

3. >= 2 of the following:
1. Renal impairment: creatinine >= 2 mg/dl for adults or >=177 mmol/L for adults
2. <= 100,000 /mm3 or <= lOOx 109/L in patients with pre-existing liver disease, a >= 2 fold elevation over baseline
3. Abnormal liver function (>= 2 upper limit of normal elevation 0fAST ALT or total bilirubin; in patients with renal disease, >= 2 fold elevation over baseline)
4. Adult respiratory distress syndrome
5. Generalized erythematous (scarlet fever) eruption + / - desquamation
6. Soft-tissue necrosis (e.g. necrotizing fascfltis, myositis, gangrene)

Front 183

Sweet’s Syndrome

-Two sets of diagnostic criteria are presented. The second set (von den Driesch Criteria) have revised the minor criteria of the first set (Su & Liu Criteria).

Back 183

In 1964, British dermatologist Robert Sweet described 8 patients with acute onset of fever, leukocytosis, painful plaqoes with a dense dermal infiltration with neutrophils histologically on the bmbs, face and neck rmder the term “acute febrile neutrophilie dermatosis

Front 184

SWEET'S SYNDROME
Su & Liu Criteria (1986)

Back 184

Diagnosis requires: Both Major criteria PLUS >= 2 Minor criteria

Front 185

Major Criteria:

Back 185

1. Abrupt onset of painful or tender erythematous or violaceous plaques or nodules
2. Predominance of neutrophulic infiltration in the dermis in the absence of leukocytoclastie vaseulitis

Front 186

Minor Criteria:

Back 186

1. Preceded by fever or infection
2. Accompanied by fever, artbralgia, conjunctivitis, or an underlying malignancy
3. Leukocytosis
4. Good Response to systemic steroids, but not to antibiotics

Front 187

SWEET'S SYNDROME
von den Driesch Criteria (I994)

Back 187

Diagnosis requires: Both Major criteria PLUS >= 2 Minor criteria

Front 188

Major Criteria:

Back 188

1. Abrupt onset of painful or tender erythematous plaques or nodules (occasionally with vesieles, pustules, or bullae)
2. Predominance of neutrophilic infiltration in the dermis in the absence of leukocytoelastic vaseulitis

Front 189

Minor Criteria:

Back 189

1. Preceded by non-speeffie respiratory or gastrointestinal tract infection or vaccination or associated with:
i. Inflammatory disease (e.g chronic autoimmune disorders, infections)
ii. Hematoproliferative disorders or solid malignant tumours, or
iii. Pregnancy
2. Accompanied by fever (>38°C) and generalized malaise
3. > 3 out of 4 abnormal laboratory values during the onset:
i. ESR>2Omm
ii. +ve C reactive protein
iii. >70% segmented nuclear neutrophils and stabs on peripheral blood smear
iv. Leukocytosis (>8,000/mm3)
4. Excellent response to treatment with systemic steroids or potassium iodide

Front 190

(SAPHO) SYNDROME

Back 190

Synovitis-Acne- Pustu losis Hyperostosis-Osteomyelitis
Syndrome

Front 191

SAPHY SYNDROME
Benhamou et al Criteria (1988)

Back 191

Diagnosis requires: >=1 criterion

Front 192

Criteria:

Back 192

1. Osteo-articular manifestations of acne conglobata, acne fulminans or hidradenitis suppurativa (i.e. inflammatory synovitis, or anterior chest wall hyperostosis or osteitis, or hyperostosis or osteitis at another site, or spondylitis-spondylodiscitis)
2. Osteo-articular manifestations of palmoplantar pustulosis (i.e. inflammatory synovitis, or pseudo-septic arthritis, hyperostosis +/- osteitis anterior chest wall, or other site, or spondylitis-spondylodiscitis)
3. Sterno-costo-clavicular, anterior chest wall, limb or spine hyperostosis +1- dermatosis
4. Chronic recurrent multifocal osteomyclitis involving the axial or peripheral skeleton + / - dermatosis

Front 193

N.B.

Back 193

• Hyperostosis is primarily an x-ray diagnosis; superficial biopsy may show bone apposition and deep biopsies, osteomyehtis.
• Anterior chest wall hyperostosis has occasionally been reported with psoriasis vulgaris or acne vulgaris.

Front 194

Exclusion criteria:The diagnosis is excluded if any of the following are present.

Back 194

1. Infectious palmoplantar pustulosis
2. Palmoplantar keratoderma
3. Infectious chest wall arthritis
4. Septic osteomyeitis
5. Diffuse idiopathic skeletal hyperostosis (DISH)
6. Osteo-articular manifestations (primarily hyperostosis) due to retinoid therapy

Front 195

Systemic Lupus Erythematosus (SLE)

Back 195

SLE
1997 Update of the 1982 ACR Revised Criteria

• SN:96%,SP:96%.1
• The diagnosis of SLE can be aided by renal or skin biopsy with characteristic histopathology

Front 196

Diagnosis requires: >= 4 of 11 criteria

Back 196

1. Malar rash: fixed erythema over malar eminences with tendency to spare nasolabial folds
2. Discoid rash
3. Photosensitivity: skin eruption as a result of unusual reaction to sunlight
4. Oral or nasopharyngeal ulcers: usually painless, observed by physician
5. Arthritis: non-erosive, affecting >=2 peripheral joints
6. Serositis: pleuritis or pericarditis
7. Renal abnormality: persistent proteinuria (>0.5 g/day or 3+) or cellular casts
8. Neurologic disorder: psychosis or seizures in the absence of offending drug or know metabolic derangements
9. Hematologic disorder: hemolytic anemia with reticulocytosis or leukopenia (>4,000mm3 on >= 2 occasions) or lymphopenia (< 1500/mm3 on >= 2 occasions), or thrombocytopenia (< 100,000/mm3)
10. Immunologic disorder: +LE prep, anti-ds DNA antibodies or anti-Sm antibodies or false positive test for syphilis positive finding of antiphospholipid antibodies*
11. Antinuclear Antibody (ANA)
*Based on:
1) Abnormal serum level of IgG or 1gM anticardiolipin antibodies
2) A positive test result for lupus anticoagulant using a standard method
3) False-positive serologic tests for syphilis known to be positive for >= 6 months and confirmed by T Pallidum immobilization or fluorescent treponemal antibody absorption test.

Front 197

Systemic Lupus Erythematosus, Drug-Induced

Back 197

DRUG INDUCED SLE
Cush & Goldings Criteria (1985)

Front 198

Diagnosis requires:

Back 198

1. No Hx of SL.E prior to drug exposure
2. >=1 features of SLE + positive ANA
3. Resolution of symptoms and eventually the ANA with drug withdrawal

Front 199

Drugs reported to induce SLE:

Back 199

Allopurinol Valproate Methyldopa
Antibiotics Antihypertensives Non-Steroidal Anti InflammatoiyAgents
Ethosuximid Captopril
Isoniazid Clonidine Phenylbutazone
Minocycline Hydralazine Piroxicam
Penicillin Practolol
Oral Contraceptives

Streptomycin Antithyroid Para-Amino Salicylic Acid
Sulfonamides Propylthiouracil Penicillamine
Tetracycline Thiamazole Phenothiazines
Anticonvulsants
Chlorpromazine Procainamide
Diphenylhydantoin Danazol Quinidine
Mephenytoin Lithium Sulfasalazine
Prirnidone Lovastatin

Front 200

N.B.

Back 200

• Rechallenge with the offending agent is not required.
• Most patients in fact meet the ACR criteria for the diagnosis of SLE, but this is not required.
• It can take up to 1 year or more for both the symptoms and serologic changes to completely resolve.
• Approximately 90% of patients with drug-induced SUE have antihistone antibodies, while 30% of idiopathic SUE have these antibodies.

Front 201

Toxic Epidermal Necrolysis (TEN)
(LyeII Syndrome)

Back 201

Toxic epidermal necrolysis is an acute life-threatening condition involving the skin and multiple mucous membranes which is most commonly drug induced. In 1956, Scottish dermatologist Alan Lyell used the term “toxic epidermal necrolysis” to describe two different diseases with extensive epidermal loss resembling scalding:TEN and Staphylococcal scalded skin syndrome
(SSSS).

Front 202

TEN
Stern & Chan Criteria (1989)

Back 202

Diagnosis is suggested by:
1. Bullae or erosions> 20% of body surface area or >= 3 different anatomic areas affected
2. Bullae or erosions on an erythematous base
3. SSSS excluded
4. Occurs on non-sun-exposed skin
5. Skin peels off in> 3cm sheets
6. Mucous membranes frequently involved
7. Tender skin within 48 hours of rash onset
8. Fever
9. Biopsy compatible with drug-inducedTEN (basal necrosis; biopsy performed within 48 hours of onset)

Front 203

Tuberous Sclerosis Complex (TSC)
(Bourneville’s disease)

Back 203

Tuberous sclerosis is an autosomal dominant neurocutaneons condition which includes the triad of epilepsy mental retardation and adenoma sebaceum. It was named by the French neurologist Dciiri-Magloire Bourneville in 1880. Two sets of criteria are presented.

Front 204

TUBEROUS SCLEROSIS
Gomez Criteria (1988)

Back 204

Diagnosis requires:

Front 205

Definite TSC:

Back 205

1 primary feature PLUS 2 secondary features

or

1 secondary feature PLUS 2 tertiary features

Front 206

Probable TSC:

Back 206

1 secondary feature PLUS 1 tertiary feature

or

3 tertiary features

Front 207

Suspect TSC:

Back 207

1 secondary feature or 2 tertiary features

Front 208

Primary features:

Back 208

1. Facial angiofibromas (histology not necessary if clinically obvious)
2. Ungual fibromas (multiple; histology not required if clinically obvious)
3. Cortical tubers (histology required)
4. Subependymal nodule or giant cell astrocytoma (histology required)
5. Calcified subependymal nodules (multiple) protruding into the ventricle (radiographic evidence)
6. Retinal astrocytomas (multiple; histology not required if clinically obvious)

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Secondary features:

Back 209

1. Shagreen patch (histology not necessary if clinically obvious)
2. Forehead plaque (histology not necessary if clinically obvious)
3. Cardiac rhabdomyoma (with radiographic or histological confirmation)
4. Cerebral tubers (radiographic evidence)
5. Non-calcified subependymal nodules (radiographic evidence)
6. Other retinal hamartomas or achromic patch (histology not necessary if clinically obvious)
7. Pulmonary lymphangiomyomatosis (histology required)
8. Renal cysts (histology required)
9. Renal angiomyolipoma (with radiographic or histological confirmation)
10. First degree relative

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Tertiary features:

Back 210

1. Confetti macules (histology not necessary if clinically obvious)
2. Hypomelanotie mactiles (histology not necessary if clinically obvious)
3. Gingival fibromas (histology not necessary if clinically obvious)
4. Enamel pits (randomly distributed) in deciduous or permanent teeth
5. Renal cysts (radiographic evidence)
6. Bone cysts (radiographic evidence)
7. Hamartomatous rectal polyps (histology required)
8. Pulmonary lymphangiomyomatosis (radiographic evidence)
9. Infantile spasms
10. Cerebral white-matter “migration tracts” or heterotopias (radiographic evidence)
11. Hamartomas of other organs (histology required)

Front 211

TUBEROUS SCLEROSIS
Roach et al Criteria (I998)

Back 211

Diagnosis requires:

Front 212

Definite TSC

Back 212

2 Major or 1 Major PLUS 2 minor criteria

Front 213

Probable TSC

Back 213

1 Major PLUS 1 Minor criterion

Front 214

Possible TSC

Back 214

1 Major or >= 2 Minor criteria

Front 215

Major Criteria:

Back 215

1. Angiofibromas on face or forehead plaque
2. Fibromas: non-traumatic ungual or periungual
3. Hypomelanotic macules (>=3)
4. Shagreen patch
5. Hamartomas: multiple retinal
6. Cortical tuber
7. Subependymal nodule or giant cell astrocytoma (histology required)
8. Multiple calcified subependymal nodules protruding into the ventricle (radiographic finding)
9. Cardiac rhabdomyoma: single or multiple
10. Lymphangiomyomatosis
11. Renal angiomyolipoma

Front 216

Minor Criteria:

Back 216

1. Dental pits: multiple, randomly distributed in dental enamel
2. Rectal polyps: hamartomatous
3. Bone cysts
4. Cerebral white matter radial migration lines
5. Gingival Fibromas
6. Hamartoma: non-renal
7. Retinal achromic patch
8. “Confetti” skin lesions
9. Renal cysts: multiple

Front 217

N.B:

Back 217

No single sign is present in all affected persons, nor is any individual clinical or radiographic sign specific forTSC.

Front 218

Vogt-Koyanagi-Harada Disease (VKHD)

Back 218

This oculocutaneous disease is characterized by vifihigo, poliosis, alopecia, bilateral uveitis with iritis and glaucoma, and dysacusia. It is named after the Japanese ophthalmologists Koyanagi and Harada, and Swiss ophthalmologist Vogt.

Front 219

VKHD
Read et al Criteria (2001)

Back 219

Diagnosis requires:

Front 220

Complete VKHD:

Back 220

Criteria #1-5 must be present

Front 221

Incomplete VKHD:

Back 221

Criteria #1-3, PLUS either #4 or 5

Front 222

Probable VKHD (isolated ocular disease):

Back 222

Criteria #1-3

Front 223

Criteria:

Back 223

1. No history of penetrating ocular trauma or surgery preceding initial onset of uveitis
2. No clinical or laboratory evidence of other ocular abnormalities
3. Bilateral ocular involvement (a or b must be met)
a. Early manifestations of disease:
i. Evidence of diffuse choroiditis (+ /- anterior uveitis, vitreous inflammatory reaction, or optic disk hyperemia), that may manifest as one of:
a. Focal area of subretinal fluid or
b. Bullous serous retinal detachments
ii. If equivocal fundus findings, both of the following must also be present:
1. Focal areas of delay in choroidal perfusion, multifocal areas of pinpoint leakage, large placoid areas of hyperfluorescence, pooling with subretinal fluid, and optic nerve staining by fluorescein angiography and
2. Diffuse choroidal thickening, without evidence of posterior scleritis on ultrasound
h. Late manifestations of disease:
i. History suggestive of prior presence of findings from 3a, and either both (ii) and (iii) below, or multiple signs from (iii)
ii. Ocular depigmentation:
1. Sunset glow fundus or 2. Sugiura sign
in. Other ocular signs:
1. Nummular chorioretinal depigmented scars or
2. Retinal pigment epithelium clumping and/or migration or
3. Recurrent or chronic anterior uveitis
4. Neurologic or Auditory findings
a. meningismus OR b. Tinnitus OR c. CSF pleocytosis
5. Findings (not preceding of CNS or ocular disease)
a. Alopecia OR b. Poliosis OR c. Vitiligo

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Wegener’s Criteria (ACR)
>2

Back 224

1. Nasal or oral Inflammation
- painful or painless oral ulcers
- pururlent or bloody nasal discharge
2. Lungs: abnormal CXR with:
- nodules
- infiltrates
- cavities
3. Kidney: urinary sediment with:
- microhematuria
- RBC casts
4. Bx: Granulomatous inflammation
- within arterial cell wall OR
- in perivascular area

Front 225

DERMATITIS HERPETIFORMIS – DIAGNOSTIC CRITERIA (Fabbri, Caproni) 2005

Back 225

The diagnosis of DH can be made on the basis of 3 main elements
1. Clinical features (pleomorphic and itchy erythematous papules, urticarial wheals and vesico-bullae predominantly located on the extensor surfaces, buttocks and back)
2. Histological picture (subepidermal bullae, eosinophil and neutrophil micro-abscessual accumulation within dermal papillae)
3. Circulating anti-endomysium (EMA) and anti tTG IgA antibodies. (these last yielding a sensitivity of 89.1% and a specificity of 97.6%)
4. DIF of perilesional skin demonstrating granular deposition of IgA in the basement membrane zone, at top of dermal papillae (sensitivity: 90%), specificity:96%)
NB:
a. Provocation or exacerbation by potassium iodide either by mouth or by patch test is not specific for DH and is now considered outmoded.
b. The diagnosis is confirmed ex juvantibus by the early and excellent response to therapy with dapsone
c. Gluten-sensitive enteropathy can be diagnosed by jejuna biopsy (villous atrophy, hyperplasia of criptae and mononuclear infiltration of the lamina propria

Front 226

1996 CDC Case Definition for Syphilis (Treponema pallidum)

Back 226

Syphilis is a complex sexually transmitted disease that has a highly variable clinical course. Classification by a clinician with expertise in syphilis may take precedence over the following case definitions developed for surveillance purposes.

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Syphilis, primary

Back 227

Clinical description: A stage of infection with Treponema pallidum characterized by one or more chancres (ulcers); chancres might differ considerably in clinical appearance.
Laboratory criteria for diagnosis: Demonstration of T. pallidum in clinical specimens by darkfield microscopy, direct fluorescent antibody (DFA-TP), or equivalent methods.
Case classification:
• Probable: a clinically compatible case with one or more ulcers (chancres) consistent with primary syphilis and a reactive serologic test (nontreponemal: Venereal Disease Research Laboratory [VDRL] or rapid plasma reagin [RPR]; treponemal: fluorescent treponemal antibody absorbed [FTA-ABS] or microhemagglutination assay for antibody to T. pallidum [MHA-TP])
• Confirmed: a clinically compatible case that is laboratory confirmed

Front 228

Syphilis, secondary

Back 228

Clinical description: A stage of infection caused by T. pallidum and characterized by localized or diffuse mucocutaneous lesions, often with generalized lymphadenopathy. The primary chancre may still be present.
Laboratory criteria for diagnosis: Demonstration of T. pallidum in clinical specimens by darkfield microscopy, DFA-TP, or equivalent methods
Case classification:
• Probable: a clinically compatible case with a nontreponemal (VDRL or RPR) titer greater than or equal to 4
• Confirmed: a clinically compatible case that is laboratory confirmed

Front 229

Syphilis, latent

Back 229

Clinical description: A stage of infection caused by T. pallidum in which organisms persist in the body of the infected person without causing symptoms or signs. Latent syphilis is subdivided into early, late, and unknown categories based on the duration of infection.
Case classification:
Probable: no clinical signs or symptoms of syphilis and the presence of one of the following:
• No past diagnosis of syphilis, a reactive nontreponemal test (i.e., VDRL or RPR), and a reactive treponemal test (i.e., FTA-ABS or MHA-TP)
• A past history of syphilis therapy and a current nontreponemal test titer demonstrating fourfold or greater increase from the last nontreponemal test titer

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Syphilis, early latent

Back 230

Clinical description: A subcategory of latent syphilis. When initial infection has occurred within the previous 12 months, latent syphilis is classified as early latent.
Case classification:
Probable: latent syphilis in a person who has evidence of having acquired the infection within the previous 12 months based on one or more of the following criteria:
• Documented seroconversion or fourfold or greater increase in titer of a nontreponemal test during the previous 12 months
• A history of symptoms consistent with primary or secondary syphilis during the previous 12 months
• A history of sexual exposure to a partner who had confirmed or probable primary or secondary syphilis or probable early latent syphilis (documented independently as duration less than 1 year)
• Reactive nontreponemal and treponemal tests from a person whose only possible exposure occurred within the preceding 12 months

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Syphilis, late latent

Back 231

Clinical description: A subcategory of latent syphilis. When initial infection has occurred greater than 1 year previously, latent syphilis is classified as late latent.
Case classification:
Probable: latent syphilis (see Syphilis, latent) in a patient who has no evidence of having acquired the disease within the preceding 12 months (see Syphilis, early latent) and whose age and titer do not meet the criteria specified for latent syphilis of unknown duration.

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Syphilis, latent, of unknown duration

Back 232

Clinical description: A subcategory of latent syphilis. When the date of initial infection cannot be established as having occurred within the previous year and the patient's age and titer meet criteria described below, latent syphilis is classified as latent syphilis of unknown duration.
Case classification:
• Probable: latent syphilis (see Syphilis, latent) that does not meet the criteria for early latent syphilis, and the patient is aged 13-35 years and has a nontreponemal titer greater than or equal to 32

Front 233

Neurosyphilis

Back 233

Clinical description: Evidence of central nervous system infection with T. pallidum
Laboratory criteria for diagnosis: A reactive serologic test for syphilis and reactive VDRL in cerebrospinal fluid (CSF)
Case classification:
Probable: syphilis of any stage, a negative VDRL in CSF, and both the following:
• Elevated CSF protein or leukocyte count in the absence of other known causes of these abnormalities
• Clinical symptoms or signs consistent with neurosyphilis without other known causes for these clinical abnormalities
Confirmed: syphilis of any stage that meets the laboratory criteria for neurosyphilis

Front 234

Syphilis, late, with clinical manifestations other than neurosyphilis (late benign syphilis and cardiovascular syphilis)

Back 234

Clinical description: Clinical manifestations of late syphilis other than neurosyphilis may include inflammatory lesions of the cardiovascular system, skin, and bone. Rarely, other structures (e.g., the upper and lower respiratory tracts, mouth, eye, abdominal organs, reproductive organs, lymph nodes, and skeletal muscle) may be involved. Late syphilis usually becomes clinically manifest only after a period of 15-30 years of untreated infection.
Laboratory criteria for diagnosis: Demonstration of T. pallidum in late lesions by fluorescent antibody or special stains (although organisms are rarely visualized in late lesions)
Case classification:
• Probable: characteristic abnormalities or lesions of the cardiovascular system, skin, bone, or other structures with a reactive treponemal test, in the absence of other known causes of these abnormalities, and without CSF abnormalities and clinical symptoms or signs consistent with neurosyphilis
• Confirmed: a clinically compatible case that is laboratory confirmed
Comment: Analysis of CSF for evidence of neurosyphilis is necessary in the evaluation of late syphilis with clinical manifestations.

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Syphilitic Stillbirth

Back 235

Clinical case definition: A fetal death that occurs after a 20-week gestation or in which the fetus weighs greater than 500 g and the mother had untreated or inadequately treated* syphilis at delivery
Comment: For reporting purposes, syphilitic stillbirths should be reported as cases of congenital syphilis.
*Inadequate treatment consists of any non-penicillin therapy or penicillin given less than 30 days before delivery.

Front 236

Names and definitions of vasculitis adopted by the Chapel Hill Consensus Conference

Back 236

Names and definitions of vasculitis adopted by the Chapel Hill Consensus Conference on the nomenclature of systemic vasculitis
1. Polyarteritis nodosa (PAN): Necrotizing inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries or venules.
2. Wegener´s granulomatosis (WG): Granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium-sized vessels, e. g. capillaries, venules, arterioles and arteries. Necrotizing glomerulonephritis is common.
3. Churg-Strauss syndrome (CSS): Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels, and associated with asthma and blood eosinophilia.
4. Microscopic polyangiitis (MSA): Necrotizing vasculitis with few or no immune deposits affecting small vessels, i.e. capillaries, venules or arterioles. Necrotizing arteritis of small and medium-sized arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs.

Front 237

ACR Criteria for the Classification of Giant-Cell Arteritis

Back 237

Three of the following five criteria were required to meet American College of Rheumatology (ACR) classification criteria for giant-cell arteritis:
1. Age 50 years or older,
2. New-onset localized headache,
3. Temporal artery tenderness or decreased temporal artery pulse,
4. Erythrocyte sedimentation rate of at least 50 mm/h, and
5. Abnormal artery biopsy specimen characterized by mononuclear infiltration or granulomatous inflammation.

These criteria have a reported sensitivity of 93.5% and a reported specificity of 91.2% for the classification of giant-cell arteritis compared with other vasculitides.

Front 238

Revised ARA Criteria for the Classification of Rheumatoid Arthritis (RA)

Back 238

For classification purposes, a patient is said to have RA if he or she has satisfied at least 4 of the following 7 criteria. Criteria 1 through 4 must have been present for at least 6 weeks. Patients with 2 clinical diagnoses are not excluded. Designation as classic, definite, or probable RA is not to be made.
1. Morning stiffness: Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement.
2. Arthritis of 3 or more joint areas: At least 3 joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician; the 14 possible joint areas are right or left proximal interphalangeal (PIP) joints, metacarpophalangeal (MCP) joints, wrist, elbow, knee, ankle, and metatarsophalangeal (MPT) joints.
3. Arthritis of hand joints: At least I area swollen (as defined above) in a wrist, MCP or PIP joint.
4. Symmetric arthritis: Simultaneous involvement of the same joint areas (see 2 above) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry).
5. Rheumatoid nodules: Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician.
6. Serum rheumatoid factor: Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in <5% of normal control subjects.
7. Radiographic changes: Radiographic changes typical of RA on posteroanterior hand and wrist radiographs, which must include erosions or unequivocal bony decalcification localized to or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify).

Front 239

Duke Criteria for Infective Endocarditis (IE)

Back 239

Clinical criteria for infective endocarditis requires:
• Two major criteria, or
• One major and three minor criteria, or
• Five minor criteria

Front 240

Major criteria:

Back 240

A. Positive blood culture for Infective Endocarditis
1- Typical microorganism consistent with IE from 2 separate blood cultures, as noted below:
• viridans streptococci, Streptococcus bovis, or HACEK* group, or
• community-acquired Staphylococcus aureus or enterococci, in the absence of a primary focus
or
2- Microorganisms consistent with IE from persistently positive blood cultures defined as:
• 2 positive cultures of blood samples drawn >12 hours apart, or
• all of 3 or a majority of 4 separate cultures of blood (with first and last sample drawn 1 hour apart)
B. Evidence of endocardial involvement
1- Positive echocardiogram for IE defined as :
• oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation, or
• abscess, or
• new partial dehiscence of prosthetic valve
or
2- New valvular regurgitation (worsening or changing of preexisting murmur not sufficient)

*HACEK group: Haemophilus sp, Actinobacilius actinomycetemcomitans, Cardiobacterium hominis, Eikenella rodens y Kingella sp

Front 241

Minor criteria:

Back 241

• Predisposition: predisposing heart condition or intravenous drug use
• Fever: temperature > 38.0° C (100.4° F)
• Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions
• Immunologic phenomena: glomerulonephritis, Osler's nodes, Roth spots, and rheumatoid factor
• Microbiological evidence: positive blood culture but does not meet a major criterion as noted above¹ or serological evidence of active infection with organism consistent with IE
• Echocardiographic findings: consistent with IE but do not meet a major criterion as noted above

Front 242

Revised Jones Criteria for Acute Rheumatic Fever (ARF)

Back 242

A firm diagnosis requires that two major or one major and two minor criteria are satisfied, in addition to evidence of recent streptococcal infection.

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Major Criteria

Back 243

1. Carditis: All layers of cardiac tissue are affected (pericardium, epicardium, myocardium, endocardium) The patient may have a new or changing murmur, with mitral regurgitation being the most common followed by aortic insufficiency.
2. Polyarthritis: Migrating arthritis that typically affects the knees, ankles, elbows and wrists. The joints are very painful and symptoms are very responsive to anti-inflammatory medicines.
3. Chorea: Also known as Syndenham´s chorea, or "St. Vitus´ dance". There are abrupt, purposeless movements. This may be the only manifestation of ARF and is its presence is diagnostic. May also include emotional disturbances and inappropriate behavior.
4. Erythema marginatum: A non-pruritic rash that commonly affects the trunk and proximal extremities, but spares the face. The rash typically migrates from central areas to periphery, and has well-defined borders.
5. Subcutaneous nodules: Usually located over bones or tendons, these nodules are painless and firm.

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Minor Criteria:

Back 244

1. Fever
2. Arthralgia
3. Previous rheumatic fever or rheumatic heart disease
4. Acute phase reactants: Leukocytosis, elevated eritrosedimentation rate (ESR) and C-reactive protein (CRP)
5. Prolonged P-R interval on electrocardiogram (ECG)

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Evidence of preceding streptococcal infection: Any one of the following is considered adequate evidence of infection:

Back 245

• Increased antistreptolysin O or other streptococcal antibodies
• Positive throat culture for Group A beta-hemolytic streptococci
• Positive rapid direct Group A strep carbohydrate antigen test
• Recent scarlet fever.

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Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome (HES)

Back 246

Diagnostic Criteria for Idiopathic Hypereosinophilic Syndrome (HES) are:
1. Persistent eosinophilia of over 1500/cubic millimeter for longer than 6 month;
2. Lack of evidence of other known causes of secondary hypereosinophilia (SH);
3. Multiple organ involvement.

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Diseases Associated with Eosinophilia

Back 247

"Allergic" Diseases
• Atopic and related diseases •Medication-related
Infectious Diseases
• Parasitic infections, mostly with helminths
• Specific fungal infections: allergic bronchopulmonary aspergillosis, coccidioidomycosis (acute and sometimes disseminated)
• Other infections--infrequent, including HIV-1 and HTLV-1
Hematologic and Neoplastic Disorders
• Hypereosinophilic syndrome • Leukemia
•Lymphomas, including nodular sclerosing Hodgkin's disease
•Tumor associated • Mastocytosis
Diseases with Specific Organ Involvement
• Skin and subcutaneous diseases, including urticaria, bullous pemphigoid, eosinophilic cellulitis (Well's syndrome), episodic angioedema with eosinophilia
• Pulmonary diseases, including acute or chronic eosinophilic pneumonia, allergic bronchopulmonary aspergillosis
• Gastrointestinal diseases, including eosinophilic gastroenteritis
• Neurologic diseases (e.g., eosinophilic meningitis)
• Rheumatologic diseases, especially Churg-Strauss vasculitis; also eosinophilic fasciitis
• Cardiac diseases (e.g., endomyocardial fibrosis)
• Renal diseases, including drug-induced interstitial nephritis, eosinophilic cystitis, dialysis
Immunologic Reactions
• Specific immune deficiency diseases: hyper-IgE syndrome, Omenn's syndrome
• Transplant rejection: lung, kidney, liver
Endocrine
• Hypoadrenalism: Addison's disease, adrenal hemorrhage
Other
• Atheroembolic disease
• Irritation of serosal surfaces, including peritoneal dialysis
• Inherited

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Diagnostic Criteria for Systemic Mastocytosis (SM)

Back 248

If at least 1 major and 1 one minor, or at least 3 minor criteria, are met, the diagnosis of Systemic Mastocytosis (SM) can be established.

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Major Criteria:

Back 249

Multifocal dense infiltrates of mast cells in bone marrow or other extracutaneous organ(s) (>15 mast cells in aggregate)

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Minor Criteria:

Back 250

a) Mast cells in bone marrow or other extracutaneous organ(s) show an abnormal morphology (> 25%)
b) C-kit mutation at codon 816 in extracutaneous organ(s). (Activating mutations at codon 816; in most cases, c-kit D816V)
c) Mast cells in bone marrow express CD2 and/or CD25
d) Serum total tryptase > 20 ng/mL (does not count in patients who have associated hematologic clonal non-mast cell lineage disease-type disease)

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ACR Criteria for the Classification of Polyarteritis Nodosa (PAN)
American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa (PAN). Classified as PAN if at least three of the 10 criteria are present:


These criteria have a reported sensitivity of 82.2% and a reported specificity of 86.6% for the classification of polyarteritis nodosa compared with other vasculitides.

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1. Weight loss > 4 kg: Loss of >4 kg body weight since illness began, not related to dieting or other factors.
2. Livedo reticularis: Mottled reticular pattern over the skin of portions of the extremities or torso.
3. Testicular pain/tenderness: Pain or tenderness of the testicles, not due to infection, trauma or other causes.
4. Myalgias, weakness or leg tenderness: Diffuse myalgias (excluding shoulder or hip girdle) or weakness of muscles or tenderness of leg muscles.
5. Mono- or polyneuropathy: Development of mononeuropathy, multiple mononeuropathies or polyneuropathy.
6. Diastolic BP >90 mmHg: Development of hypertension with the diastolic BP higher than 90 mmHg.
7. Elevated BUN or creatinine: Elevation of BUN >40 mg/dl or creatinine >1.5 mg/dl, not due to dehydration or obstruction.
8. Hepatitis B virus: Presence of hepatitis B surface antigen or antibody in serum.
9. Arteriographic abnormality: Arteriogram showing aneurysms or occlusions of the visceral arteries, not due to arteriosclerosis, fibromuscular dysplasia or other non-inflammatory causes.
10. Biopsy of small or medium-sized artery containing polymorphonuclear cells: Histologic changes showing the presence of granulocytes or granulocytes and mononuclear leucocytes in the artery wall.

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Amsel's Diagnostic Criteria for Bacterial Vaginosis

Three of four criteria must be met; establishes accurate diagnosis of bacterial vaginosis in 90 percent of affected women.

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• Homogeneous vaginal discharge (color and amount may vary)
• Amine (fishy) odor when potassium hydroxide solution is added to vaginal secretions (commonly called the "whiff test")
• Presence of clue cells (greater than 20%) on microscopy*
• Vaginal pH greater than 4.5
* Highly significant criterion.

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Criteria used to associate a dermatosis and malignancy (curth's postulates).

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Concurrent Onset: The neoplasm is discovered at the time of diagnosis of the dermatosis, or shortly following diagnosis

Parallel Course: Therapy of the malignancy results in a disappearance of the dermatosis, and, if the malignancy recurs, then the dermatosis relapses

Uniform site or type: The neoplasm is of a specific cell type within a specific of neoplasm organ or tissue

Statistical association

Genetic linkage

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Acquired generalized lipodystrophy: proposed diagnostic criteria and subtypes

Back 254

Essential criterion:

Selective loss of fat involving large regions of the body beginning after birth (usually by adolescence)

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Supportive criteria

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Clinical

• Loss of subcutaneous fat from the palms and soles
• Acanthosis nigricans
• Hepatosplenomegaly
• Panniculitis prior to onset (by clinical history or histologic confirmation; see below)
• Associated autoimmune diseases (see below)

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Supportive criteria

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Laboratory

• Diabetes mellitus or impaired glucose tolerance
• Severe hyperinsulinemia (fasting and/or postprandial)
• Increased serum triglyceride and/or decreased HDL cholesterol levels
• Reduced serum leptin and/or adiponectin levels
• Anthropomorphic or MRI evidence of large regions of fat loss
• MRI evidence of preserved bone marrow fat

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Subtypes

Back 257

Panniculitic variant (type 1)

• Preceding panniculitis; lipoatrophy may be noted upon its resolution, in the center of expanding annular lesions or at distant sites
• Mean age of onset is 7 years; slight female predominance (∼1.5-fold)
• Associ
ted symptoms include low-grade fever, malaise, arthralgias and abdominal pain
• May be associated with autoimmune diseases (see below)
• Relatively mild metabolic derangements and leptin abnormalities
• Histopathologic evaluation reveals a lymphohistiocytic subcutaneous infiltrate ± granulomatous foci

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Subtypes

Back 258

Autoimmune variant (type 2)

• Concurrent or prior autoimmune disease without preceding panniculitis; associated with juvenile dermatomyositis, Sjögren's syndrome > juvenile
diopathic arthritis, vitiligo, chronic urticaria/angi↓dema and autoimmune thyroiditis, hepatitis or hemolytic anemia
• Mean age of onset is 15 years; female predominance (∼3-fold)
• Hepatomegaly is invariably present; hypertriglyceridemia
nd diabetes mellitus occur in most patients
• Laboratory abnormalities may be observed without clinical evidence of autoimmune disease, including antinuclear, anti-smooth muscle, anti- glomerular basement membrane, anti-salivary duct, antimitochondrial and anti-adrenal/ovary/placenta/testis antibodies

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Subtypes

Back 259

Idiopathic (type 3)

• No evidence of panniculitis or autoimmune disease
• Mean age of onset is 20 years (range, <2–60 years); female predominance (∼3-fold)

Front 260

Diagnostic criteria of scleromyxedema versuslocalized variants of lichen myxedematosus

Back 260

Scleromyxedema

1. Generalized papular eruption andsclerodermoid features
2. Microscopic triad (mucin deposition, fibroblast proliferation, fibrosis)
3. Monoclonal gammopathy
4. Absence of thyroid disorder


Localized variants of lichenmyxedematosus

1. Papular eruption (or nodules and/orplaques due to confluence of papules)
2. Mucin deposition with variablefibroblast proliferation
3. Absence of monoclonal gammopathy
4. Absence of thyroid disorder

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Histologic criteria used to define amyloid

Back 261

• Homogeneous, hyalin, eosinophilic deposits in H&E-stained sections
• Crystal violet metachromasia
• Positive staining with alkaline Congo red
• Apple green birefringence under polarized light after Congo red staining
• Fibrillar structure on electron microscopy
• Staining with antibodies to amyloid P component
• Staining with antibodies directed against specific precursors

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Criteria that point to the diagnosis of Buerger's disease

Back 262

1. history of smoking
2. an age <50 years
3. an absence of atherosclerotic risk factors other than smoking
4. infrapopliteal occlusions, upper limb involvement and migratory thrombophlebitis

Front 263

Diagnostic criteria and classification of hypereosinophilic syndromes (HES).

Back 263

DIAGNOSTIC CRITERIA

• Peripheral blood eosinophil counts > 1500/ml for at least 6 months, or less than 6 months with evidence of organ damage
• Lack of evidence for parasitic, allergic, or other recognized causes of eosinophilia
• Symptoms and signs of organ system involvement

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Subtypes

Back 264

Myeloproliferative[*] Includes patients with eosinophilic leukemia, who may have other cytogenetic abnormalities

• FIP1L1-PDGFRA fusion gene; other as yet unfound mutations are possible
• Includes ‘classic HES' with endomyocardial disease and a male predominance
• Mucosal ulcers are associated with severe disease
• High serum tryptase and vitamin B12 levels, tissue fibrosis, splenomegaly, and bone marrow biopsies with increased numbers of CD25+ atypical spindle-shaped mast cells[†] In the WHO classification of mastocytosis, such patients (who have the FIP1L1-PDGFRA fusion gene) are designated as having systemic mastocytosis with associated clonal hematological non-mast celllineage disease (AHNMD).

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Subtypes

Back 265

Lymphoproliferative

• T-cell clone producing Th2 cytokines
• T-cell clones have occurred in rare cases of EAE and NERDS

Front 266

Subtypes

Back 266

Other[‡] Other HES subtypes have recently been reviewed and partially classified

• Eosinophilic vasculitis
• Episodic angioedema with eosinophilia (EAE)
• Nodules, eosinophilia, rheumatism, dermatitis and swelling (NERDS) syndrome
• A variety of other diseases, including eosinophilic gastroenteritis

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Classification of mastocytosis

Back 267

Classification based on clinical criteria

Type Ia - Indolent mastocytosis without systemic disease

Type Ib - Indolent mastocytosis with systemic disease

Type II[*] - Mastocytosis associated with a myeloproliferative or myelodysplastic disease
(Includes patients with the FIP1L1-PDGFRA mutation in association with hypereosinophilic syndrome/chronic eosinophilic leukemia)

Type III - Lymphadenopathic mastocytosis (with eosinophilia) (Patients often do not have cutaneous lesions)

Type IV - Mast cell leukemia
(Patients often do not have cutaneous lesions)

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CLASSIFICATION OF MASTOCYTOSIS

Back 268

WHO classification

Ia. Cutaneous mastocytosis

Ib. Indolent systemic mastocytosis

II. Systemic mastocytosis with associated clonal hematological non-mast cell-lineage disease (AHNMD)

IV. Aggressive systemic mastocytosis

V. Mast cell leukemia

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WHO criteria for the diagnosis of systemic mastocytosis

Back 269

Requires either the major criterion plus one minor criterion or three minor criteria

Major criterion
• Multifocal dense in.ltrates of mast cells (aggregates of >15 mast cells) in bone marrow or extracutaneous tissues

Minor criteria
• >25% of mast cells in bone marrow samples or extracutaneous tissues are spindle-shaped or otherwise atypical
• Extracutaneous mast cells (CD117+) express CD2, CD25 or both (determined via flow cytometry)
• Presence of c-kit codon 816 mutation in blood, bone marrow or extracutaneous tissues
• Serum total tryptase level is persistently >20 ng/ml (except in AHNMD)

Front 270

Mastocytosis Classification based on c-kit mutations detected to date

Back 270

Childhood disease

1. No activating mutation (most)
2. 816 activating mutation (few)[‡] - For example, when it leads to an Asp/Val substitution, the abbreviation is D816V, and when it leads to an Asp/Phe substitution, D816F.
3. Inactivating mutation (e.g. E839K) (few)


Adult disease
1. 816 activating mutation (most)[‡] - For example, when it leads to an Asp/Val substitution, the abbreviation is D816V, and when it leads to an Asp/Phe substitution, D816F.
2. Other activating mutations (e.g. F522C[§], V560G, D820G) (few) - Disease responsive to imatinib; an F522C germline mutation was described in a woman with adult-onset systemic mastocytosis and a history of childhood urticaria pigmentosa that resolved by adolescence.

Familial disease
1. No activating or inactivating mutation (most)
2. Activating mutations (e.g. K509I, A533D) (few)

Familial gastrointestinal stromal tumors (GISTs) plus hyperpigmentation cell disease
1. Activating mutations (e.g. deletion of V559 & V560; V559A; L576_P577insQL; deletion of D419)

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POEMS

Back 271

(polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome: Criteria for diagnosis

Front 272

POEMS

Back 272

Cutaneous manifestations: including hyperpigmentation, hypertrichosis, hemangiomas (cherry hemangiomas and the characteristic glomeruloid), acrocyanosis, flushing, and leukonychia, dermatologists may be the first to think of the diagnosis. Patients must have polyneuropathy and a plasma cell dyscrasia and at least one other major criterion and one minor criterion (Table 1).

Front 273

POEMS

MAJOR CRITERIA

Dx of monoclonal gammopathy =SPEP and immunofixation electrophoresis.

Back 273

1. Polyneuropathy
2. monoclonal plasma cell-proliferative disorder (almost always lambda)
3. sclerotic bone lesions
4. Castleman disease
5. VegF elevation

Front 274

POEMS

MINOR CRITERIA

Back 274

6. Oranomegaly (spleen, liver, lymph)
7. Extravascular volume overload (edema, pleural effusion, ascites)
8. Endocrinopathy (adrenal, thyroid†, pituitary, gonadal, parathyroid, pancreatic†)
9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomas, plethora, acrocyanosis, flushing, white nails)
10. Papilledema
11. Thrombocytosis/polycythemia‡
(Anemia and thrombocytopenia are distinctively unusual in this syndrome unless Castleman disease is present)

Front 275

POEMS

OTHER SIGNS AND SYMPTOMS

Back 275

Clubbing, weight loss, hyperhidrosis, pulm HTN/ restr. Lung dz, thrombotic diatheses, diarrhea, low vitamin B12

Front 276

POEMS

POSSIBLE ASSOCIATIONS

Back 276

Arthralgias, cardiomyopathy (systolic dysfun) and fever

Front 277

ERYTHROMELALGIA

Back 277

The diagnostic criteria:

(1) burning pain in the extremities;
(2) pain aggravated by warming;
(3) pain relieved by cooling;
(4) erythema of affected skin; and
(5) increased temperature of affected skin

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EM Minor

Back 278

Skin Lesions
• Typical targets
• ± Papular atypical targets

Distribution
Extremities (especially elbows, knees, wrists, hands), face

Mucosal Involvement
Absent or mild

Systemic Sx
Absent

Progression to TEN
No

Precipitating Factors
• Herpes simplex virus
• Other infectious agents

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EM Major

Back 279

Type of Skin Lesions
• Typical targets
• ± Papular atypical targets
• Occasionally bullous lesions


Distribution
Extremities, face

Mucosal Involvement
Severe

Systemic Sx
Present

Progression to TEN
No

Precipitating Factors
• Herpes simplex virus
• Mycoplasma pneumoniae
• Other infectious agents
• Rarely drugs

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SJS

Back 280

Type of Skin Lesions
• Dusky macules with or without epidermal detachment
• Macular atypical targets
• Bullous lesions (<10% BSA detachment)

Distribution
Trunk, face

Mucosal Involvement
Severe

Systemic Sx.
Present

Progression to TEN
Possible

Precipitating Factors
• Drugs

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DSM-IV diagnostic criteria for trichotillomania

Back 281

1. Recurrent pulling out of one's hair resulting in noticeable hair loss
2. An increasing sense of tension immediately before pulling out the hair or when attempting to resist the behavior
3. Pleasure, gratification or relief when pulling out the hair
4. The disturbance is not better accounted for by another mental disorder and is not due to a general medical condition (e.g. a dermatologic condition)
5. The disturbance provokes clinically marked distress and/or impairment in occupational, social or other areas of functioning

Front 282

Smallpox

Variola/Orthopoxvirus
Humans

Back 282

Major criteria*

• Febrile prodrome (>101°F[†], 1–4 days prior to rash onset with headache, backache or abdominal pain)
• Firm, deep-seated, well-circumscribed vesicles/pustules
• Lesions in the same stage of development in any one area of the body

Minor criteria
• Centrifugal distribution[‡]

• First lesions in the pharynx, oral mucosa
• Patient appears ‘toxic’
• Slow evolution of rash (i.e. 1–2 days for each stage: macule, papule, vesicle)
• Lesions on the palms and soles

Front 283

CURRENTLY ACCEPTED MAJOR AND MINOR EPIDERMOLYSIS BULLOSA TYPES AND SUBTYPES

Back 283

EB simplex (EBS)
Junctional EB (JEB)
Dystrophic EB (DEB)

Front 284

EB simplex (EBS)
Major EB subtypes

Back 284

Major EB subtypes

EBS, Weber–Cockayne (EBS-WC)
EBS, Koebner (EBS-K)
EBS, Dowling–Meara (EBS-EBS with muscular dystrophy (EBS-MD)

Front 285

EB simplex (EBS)
Minor EB subtypes

Back 285

Minor EB subtypes

EBS with mottled pigmentation (EBS-MP)
Autosomal recessive EBS (EBS-AR)
EBS superficialis (EBSS)
EBS, Ogna
EBS with pyloric atresia (EBS-PA)

Front 286

Junctional EB (JEB)

Back 286

Major EB subtypes

JEB, Herlitz (JEB-H)
JEB, non-Herlitz (JEB-nH)[*]
JEB with pyloric atresia (JEB-PA)

Front 287

Junctional EB (JEB)

Back 287

Minor EB subtypes

JEB inversa (JEB-I)
JEB, localized (JEB-lo)

Front 288

Dystrophic EB (DEB)

Back 288

Major EB subtypes

Dominant DEB (DDEB)
Recessive dystrophic EB, Hallopeau–Siemens (RDEB-HS)
RDEB, non-Hallopeau–Siemens (RDEB-nHS)

Front 289

Dystrophic EB (DEB)

Back 289

Minor EB subtypes

DDEB, pretibial (DDEB-Pt)
DDEB pruriginosa (DDEB-Pr)
RDEB inversa (RDEB-I)
RDEB centripetalis (RDEB-Ce)
DEB, transient bullous dermolysis of the newborn (DEB-TBDN)[†]
DEB, autosomal dominant/autosomal recessive heterozygote

Front 290

A recent study found that, in patients with a subepidermal blistering disorder associated with linear deposits of IgG or C3 along the epidermal basement membrane, the presence of the following four clinical criteria strongly indicated a diagnosis of BP:

Back 290

• absence of skin atrophy
• absence of mucosal involvement
• absence of head and neck involvement
• age greater than 70 years.

Front 291

Dermoscopic criteria and their corresponding histopathologic features.

Back 291

Criterion

Morphological definition

Associated histopathologic changes

Diagnosis

Front 292

Pigment network

Back 292

Network of brownish lines over a diffuse tan background

Pigmented rete ridges

Melanocytic lesion

Front 293

Typical network

Back 293

Brown-colored, regularly meshed and narrowly spaced network

Regular and elongated rete ridges

Benign melanocytic lesion

Front 294

Atypical network

Back 294

Black, brown or gray network with irregular meshes and thick lines

Irregular and broadened rete ridges

Melanoma

Front 295

Dots/globules

Back 295

Black, brown and/or gray, round to oval, variably sized structures regularly or irregularly distributed within the lesion

Pigment aggregates within the stratum corneum, epidermis, dermo-epidermal junction or papillary dermis

If regular: benign melanocytic esion; if irregular: melanoma

Front 296

Streaks

Back 296

Irregular, linear structures not clearly combined with pigment network lines at the margins

Confluent junctional nests of melanocytes

Melanoma

Front 297

Blue-whitish veil

Back 297

Irregular, confluent, gray-blue to whitish-blue diffuse pigmentation

Acanthotic epidermis with focal hypergranulosis above sheets of heavily pigmented melanocytes in the dermis

Melanoma

Front 298

Blotches

Back 298

Black-, brown- and/or gray-colored areas with regular or irregular shape/distribution

Hyperpigmentation throughout the epidermis and/or upper dermis

If regular: benign melanocytic lesion; if irregular: melanoma

Front 299

Regression structures

Back 299

White (scar-like) areas, blue (pepper-like) areas or combinations of both

Thickened papillary dermis with fibrosis and/or variable amounts of melanophages

Melanoma

Front 300

Milia-like cysts

Back 300

White-yellowish, roundish dots

Intraepidermal horn globules, also called horn pseudocysts

Seborrheic keratosis (occasionally observed in papillomatous melanocytic nevi)

Front 301

Comedo-like openings

Back 301

Brown-yellowish, round to oval or even irregularly shaped, sharply circumscribed structures

Keratin plugs situated within dilated follicular openings
Seborrheic keratosis

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Leaf-like areas

Back 302

Brown-gray to gray-black patches revealing a leaf- like configuration

Pigmented, solid aggregations of basaloid cells in the papillary dermis

Basal cell carcinoma

Front 303

Red-blue lacunas

Back 303

Sharply demarcated, roundish to oval areas with a reddish, red-bluish, or red-black color

Dilated vascular spaces situated in the upper dermis

Vascular lesion

Front 304

Vascular structures

Back 304

Comma-like vessels

Arborizing vessels

Hairpin vessels

Dotted or irregular vessels



Benign melanocytic lesion
Basal cell carcinoma
Seborrheic keratosis
Melanoma

Front 305

Criteria for the diagnosis of true idiopathic aquagenic pruritus

Back 305

(1) severe pruritus occurring after water contact, irrespective of water temperature or salinity; (2) pruritus developing within minutes of water contact without visible skin changes, i.e. in the absence of urticaria or symptomatic dermatographism; and (3) exclusion of chronic skin diseases, drug-related pruritus, and systemic disorders (e.g. polycythemia vera and myeloproliferative disorders)

Front 306

Schnitzler's syndrome

Back 306

urticarial vasculitis in the setting of a monoclonal IgM gammopathy plus at least two of the following: fever, arthralgias or arthritis, bone pain, lymphadenopathy, hepatosplenomegaly, an elevated ESR, leukocytosis, or abnormal findings on bone morphologic investigations.

Front 307

International Consensus Statement–preliminary criteria for antiphospholipid antibody syndrome

Back 307

Definite diagnosis requires at least one clinical and one laboratory criterion.

Front 308

Clinical criteria

Back 308

1. Vascular thrombosis
• One or more clinical episodes of arterial, venous or small vessel thrombosis

2. Complications of pregnancy
• One or more unexplained deaths of morphologically normal fetuses at or after the 10th week of pregnancy; or
• One or more premature births of morphologically normal neonates at or before the 34th week of gestation; or
• Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation

Front 309

Laboratory criteria

Back 309

1. Anticardiolipin antibodies[*], IgG or IgM, present at moderate or high levels[†] on two or more occasions at least 12 weeks apart
2. Lupus anticoagulant antibodies on two or more occasions at least 12 weeks apart
3. Anti-b2-glycoprotein I antibodies, IgG or IgM (in titer >99th percentile) on two or more occasions at least 12 weeks apart

* β2-glycoprotein I-dependent.
† Several thresholds exist for low versus moderate-to-high: (1) >40 international ‘phospholipid’ units; (2) 2–2.5× the median level of anticardiolipin antibodies (ACA); and (3) 99th percentile for ACA in the normal population.

Front 310

• autologous transplantationselection criteria for Vitiligo

Back 310

1)stable vitiligo (for at least 6 months),
2) unsatisfactory response to medical therapy,
3)absence of Koebner phenomena,
4) positive minigrafting test,
5) no tendency for scar or keloid formation, and
6)age above 12 years.

Front 311

CRITERIA FOR THE DIAGNOSIS OF PRIMARY HYPERHIDROSIS

Back 311

1. Focal, visible excess sweating

2. Present for at least 6 months

3. No apparent secondary causes
At least 2 of the following:
• Bilateral and symmetric
• Impairs activities of daily life
• At least 1 episode per week
• Age of onset <25 years
• Positive family history
• Stops during sleep
severely compromised socially and at work or school
right axilla usually produces more sweat than the left (60-40).
Odor (axillary bromhidrosis) is usually absent

Front 312

atypical melanocytic nevi undoubtedly encompass a large and heterogeneous group of nevi

Back 312

• nevi with atypical clinical features, which have been termed simply ‘atypical nevi’; in general, these atypical nevi can simulate melanoma; this group of nevi should include not only ‘atypical’ or ‘dysplastic’ nevi, but also some congenital and combined melanocytic, as well as Spitz/pigmented spindle cell nevi;
• nevi with abnormal histopathologic features;
• nevi with both abnormal clinical and histopathologic features;
• nevi with histopathologic features that are equivocal or of unknown significance.

Front 313

DIAGNOSTIC CLINICAL FEATURES OF PSORIATIC ARTHRITIS

Back 313

A. CHARACTERISTIC FEATURES
a. Psoriasis present
b. Enthesitis
c. Dactylitis
d. Onychodystrophy
e. DIP joint involvement
f. Juxta-articular new bone formation
g. Sacroilitis and/or spondyloarthritis
h. Isolated involvement of the joints of one ray (eg. DDIP, PIP, monocyte chemotactic protein)
i. Insidious appearance of ankylosed joins (eg. Hallux rigidus)
B. SUPPORTING FEATURES
a. In the absence of documented psoriasis, a convincing history of psoriasis and/or a family history positive for psoriasis
b. Peripheral arthritis, often asymmetrical or oligoarticular at onset
C. EXCLUSIONS
a. Fibromyalgia
b. Seronegative or seropositive rheumatoid arthritis
c. Intercurrent arthritides (eg. Lyme disease)
d. Repetitive motion – induced musculoskeletal syndromes

Front 314

ALGORITHM FOR THE DIAGNOSIS OF PATCH STAGE MYCOSIS FUNGOIDES (4 POINTS ARE REQUIRED)
- For clinical diagnosis, patients presenting with >= 4 points are considererd to have unequivocal MF

Back 314

CLINICAL (1 or 2 points): Persistent, progressive patches and plaques +/-
a. non sun exposed distribution
b. Variation in size and shape
c. Poikiloderma
HISTOPATHOLOGIC (1 or 2 points): Superficial dermal T-cell infiltrate +/-
a. Epidermotropism
b. Nuclear atypia
IMMUNOPATHOLOGIC (1 point only):
a. CD2, CD3, or CD5 <50%
b. CD7<10%
c. Epidermal – dermal discordance
MOLECULAR BIOLOGIC (1 point only)
a. Dominant T cell clonality

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DIAGNOSTIC CRITERIA FOR CLASSIC SWEET SYNDROME

- Both major criteria and 2 of 4 minor criteria is required to establish diagnosis of classi Sweet syndrome

Back 315

MAJOR
a. Abrupt onset of painful erythematous plaques or nodules
b. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis
MINOR
1. PYREXIA >38 C, 100 F
2. Association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, or preceded by an URTI or GI infection or vaccination
3. Excellent response to treatment with systemic corticosteroids or potassium iodide
4. Abnormal laboratory values at presentation (3 of 4): ESR>20 mm/h, positive CRP, >8000 leukocytes, >70% neutrophils

Front 316

DIAGNOSTIC CRITERIA FOR DRUG INDUCED SWEET SYNDROME

-All five criteria are required for the diagnosis of drug induced Sweet Syndrome

Back 316

1. abrupt onset of painful erythematous plaques or nodules
2. histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis
3. Pyrexia >38 C
4. Temporal relationship between drug ingestion and clinical presentation, or temporally related recurrence after oral challenge
5. Temporally related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids

Front 317

DIAGNOSTIC CRITERIA OF PYODERMA GANGRENOSUM

Back 317

The diagnosis of PG is likely in a patient with:
1. Sudden onset of a painful lesion fitting the morphologic criteria (ulcerative, bullous, pustular, or vegetative) in a (usually middle aged) patient without significant toxaemia or relevant drug intake
AND
2. Histologic evidence of marked tissue neutrophilia in the absence of leudcoytoclastic vasculitis and exclusion of malignancy and infective organisms by special studies and negative tissue culture
AND
3. Exclusion of vascular stasis/occlusion/vasculitis by appropriate studies
The following minor criteria provide supportive evidence of the diagnosis or raise suspicion of PG in patients who do not fulfil all the major criteria above:
1. Localization at a characteristic site (ulcerative PG on the legs, vegetative on the trunk, bullous PG on the upper limb, pustular PG on the truck or face) ra at a site of cutaneous trauma (ulcerative PG) or in a peristomal site
2. Rapid progression of the inflamamatory lesion with escalating pain severity (except vegetative PG)
3. Occurrence in an individual with systemic disease, such as arthritis, inflammatory bowel disease, or hematologic dyscrasia (except vegetative PG)
4. Rapid reduction of pain and inflammation on initiation of systemic steroid therapy

Front 318

DIAGNOSTIC CRITERIA FOR HYPEREOSINOPHILIC SYNDROME

Back 318

1. Peripheral blood eosinophilia of at lease 1500 eos/uL
a. Longer than 6 mo OR
b. Less than 6 mo with evidence of organ damage
2. Signs and symptoms of multiorgan involvement
3. No evidence of parasitic or allergic disease or other known causes of peripheral blood eosinophilia

Front 319

CRITERIA FOR IRRITANT CONTACT DERMATITIS (RIETSCHEL)
MAJOR

Back 319

1. SUBJECTIVE
a. Onset of symptoms within minutes to hours of exposure
b. Pain, burning, stinging, or discomfort exceeding itching early in the clinical course
2. OBJECTIVE
a. Macular erythema, hyperkeratosis, or fissuring predominating over vesiculation
b. Glazed, parched, or scalded appearance of the epidermis
c. Healing process begins promptly on withdrawal of exposure to the offending agent
d. Patch testing is negative

Front 320

MINOR

Back 320

1. Onset of dermatitis within 2 weeks of exposure
2. Many people in the environment affected similarly
3. Sharp circumspection fo the dermatitis
4. Evidence of gravitational influence, such as dripping effect
5. Lack of tendency of the dermatitis to spread
6. Morphologic changes suggestingsmall concentration differences or contact time produce large differences in skin changes

Front 321

DIAGNOSTIC CRITERIA FOR EPIDERMOLYSIS BULLOSA ACQUISITA (Yaoita)

Back 321

1. A bullous disorder within the clinical spectrum outlined earlier (see clinical findings)
2. No family history of a bullous disorder
3. Histology showing a sub-epidermal blister
4. Deposition of immunoglobulin G deposits within the dermal-epidermal junction (i.e. a positive direct immunofluorescence of perilesional skin)
5. Immunoglobulin G deposits localized to the lower lamina densa and/or sub-lamina densa zone of the dermal-epidermal junction when perilesional skin is examined by direct immunoelectron microscopy

Front 322

DIAGNOSTIC CRITERIA WAARDENBURG SYNDROME TYPE 2

- 2 / 4 criteria, in the absence of dystopia canthorum, limb deformity, or Hirschsprung disease, should be counted as affected

Back 322

1. Congenital sensorineural hearing loss
2. Pigmentary disturbance of iris
a. Complete heterochromia irides
b. Partial or segmental heterochromia (segments of blue or brown pigmentation in one or both eyes)
c. Hypoplastic blue eyes (characteristic brilliant blue in both eyes)
3. Pigmentary disturbance of the hair
a. White forelock from birth or in teens
b. Premature graying before age 30
4. A first or 2nd degree relative with 2 or more criteria of 1 to 3

Front 323

CLINICAL SIGNS THAT REQUIRE HISTOLOIGC EVALUATION OF LONGITUDINAL MELANONYCHIA

Back 323

1. Periungual pigmentation
2. Adult age
3. Change in color/width of the band
4. Hyperpigmented lines within the band
5. Proximal portion of the band wider than distal
6. Thumb, index finger, or to involvement
7. Blurred margins
8. History of trauma (negative prognostic factors)

Front 324

AMERICAN BURN ASSOCIATION BURN CENTER TRANSFER CRITERIA

Back 324

1. 2nd and 3rd degree burns >10% TBSA in patients younger than 10 or over 50 years of age
2. 2nd and 3rd degree burns greater than 20% BSA in other age groups
3. 2nd and 3rd degree burns that involve the face, hands, feet, genitalia, perineum and major joints
4. 3rd degree burns greater than 5% BSA in any age group
5. Electrical burns, including lightning injury
6. Chemical burns
7. Inhalation injury
8. Burn injury in patients with pre-existing medical disorders that could complicate management, prolong recovery , or affect mortalit
9. Any patients with burns and concomitant trauma (such as fractures, etc.) in which the burn injury poses the greatest risk of morbidity or mortality. In such cases, if the trauma poses the greater immediate risk, the patient may be treated initially in a trauma center until stable before being transferred to a burn center. Physician judgement will be necessary in such a situation and should be in concert iwth the regional medical control plan and triage protocols.
10. Hospitals without qualified personnel or equipment for the care of children should transfer children with burns to a burn center with these capabilities
11. Burn injury in patients who will require special social/emotional and/or long-erm rehabilitative support, including cases involving suspected childl abuse, substance abuse

Front 325

DIAGNOSTIC CRITERIA FOR BASAL CELL NEVUS SYNDROME (Kimonis)

Back 325

MAJOR CRITERIA
1. Basal cell ca before the age of 20
2. Odontogenic keratocysts before age 15
3. Three or more palmar and or plantar pits
4. Bilamellar calcification of the falx cerebri (if younger than 20 year_
5. Fused, bifid, or markedly splayed ribs
6. First-degree relative with bcns
7. PTC gene mutation in normal tissue
MINOR CRITERIA
1. Marcocephaly determined after adjustment for height
2. Congenital malformations: cleft lip or palate, frontal bossing, “coarse face”, moderate or severe hypertelorism
3. Skeletal abnormalities: sprengel deformity, marked pectus deformilty, or marked syndactyly of the digits
4. Radiologic abnormalities: bridging of the sella turcica; rib anomalies such as bifid or splayed ribs; vertebral anomalies such as hemivertebrae, fusion, or elongation of the vertebral bodies; modelling defects of the hands and feet; or flame shaped lucencies of the hands and feet
5. Ovarian fibroma
6. Medulloblastoma

Front 326

DIAGNOSTIC CRITERIA OF DYSPLASTIC NEVUS

Back 326

- 2 obligate features
1. Diameter in one dimension at least 5 mm
2. Prominent flat component
- 2 / 3 other features
1. Irregular asymmetric outline
2. Indistinct borders
3. Variable pigmentation

Front 327

BEIGHTON CRITERIA FOR JOINT HYPERMOBILITY

-numbers 1-4 are scored fore each side, so that a maximal score of two is possible if both left and right sides show criteria; number 5 is scored as 1. A score of >=5/9 is hypermobile

Back 327

1. Passive dorsiflexion of the fifth finger >90 degrees
2. Passive apposition of the thums to the flexor aspect of the forearm (Beighton’s sign)
3. Hyperextension of the elbow >10 degrees
4. Hyperextension fo the knees >10 degrees
5. Ability of the palms to completely touch the floor during forward flexion of the trunk with the knees fully extended

Front 328

DIAGNOSTIC CRITERIA FOR TUBEROUS SCLEROSIS COMPLEX – GOMEZ REVISED CRITERIA



DIAGNOSTIC CRITERIA FOR TUBEROUS SCLEROSIS COMPLEX – GOMEZ REVISED CRITERIA

Back 328

Definite TSC = presence of either two major features or one major feature and two minor features
Probable TSC = presence of one major feature or two or more minor features
* implies cutaneous and oral lesions

Front 329

MAJOR FEATURES

Back 329

1. Facial angiofibromas or forehead plaque*
2. Non-traumatic ungula or periungual fibroma*
3. Hypomelanotic macules (three or more)*
4. Shagreen patch (connective tissue nevus)*
5. Multiple retinal nodular hamartoma
6. Cortical tuber – when cerebral cortical dysplasia and cerebral white matter migration tracts occur together, they should be counted as one rather than two features of TSC
7. Subependymal nodule
8. Subpendymal giant cell astrocytoma
9. Cardiac rhabdomyoma, single or multiple
10. Lymphangiomyomatosis – when both lymphangiomyomatosis and renal angiomyolipomas are present, other features of TSC should be present before a definitive diagnosis is assigned
11. Renal angiomyolipoma

Front 330

MINOR FEATURES

Back 330

1. Multiple randomly distributed pits in dental enamel*
2. Hamartomatous rectal polyps – histologic confirmation is suggested
3. Bone cysts
4. Cerebral white matter migration lines – radiographic confirmation is sufficient
5. Gingival fibromas*
6. Non-renal hamartoma – histologic confirmation is suggested
7. Retinal achromic patch
8. Confetti skin lesions*
9. Multiple renal cysts – histologic confirmation is suggested

Front 331

DIAGNOSTIC CRITERIA FOR NEUROFIBROMATOSIS TYPE 1 (NF-1)

Back 331

- 2 or more required for diagnosis

Front 332

CRITIERIA

Back 332

1. 6 or more cafe au lait macules larger than 5 mm in greatest diameter in prepubertal individuals and over 15 mm in post-pubertal individuals
2. Two or more neurofibromas of any type OR one plexiform neurofibroma
3. Freckling in the axillary or inguinal regions
4. Optic glioma
5. 2 or more iris Lisch nodules
6. A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex with or without pseudarthrosis
7. A first-degree relative (parent, sibling, or offspring) with NF-1 by the above criteria

Front 333

MANCHESTER CLINICAL DIAGNOSTIC CRITERIA FOR NEUROFIBROMATOSIS Type II (NF-2)

Back 333

-A patient has the clinical diagnosis of NF-2 if one of the following criteria is present:
1. bilateral vestibular schwannomas
2. first-degree family relative with NF-2 and unilateral vestibular schwannoma or any two of the following: meningioma, schwannoma, glioma, neurofibroma, or posterior subcapsular lenticular opacities
3. Unilateral vestibular schwannoma and any two of the following: meningioma, schwannoma, glioma, neurofibroma, or posterior subcapsular lenticular opacities
4. Multiple meningiomas (two or more) and unilateral vestibular schwannoma and any two of the following: schwannoma, glioma, neurofibroma, cataract

Front 334

CRITERIA FOR POLYCYTHEMIA VERA

Back 334

1. Elevated red cell mass
2. Normal arterial O2
3. Splenomegaly,. In the absence of splenomegaly, two of the following minor criteria must occur:
a. Leukocytosis more than 12000/uL
b. Thrombocytosis >400000/uL
c. Leukocyte alkaline phosphatise >100 units
d. Vitamin B12 >900 pg/mL

Front 335

CLASS I HISTIOCYTOSIS (LCH) – THE WRITING GROUP OF THE HISTIOCYTE SOCIETY

Back 335

1. Presumptive diagnosis – light morphologic characteristics
2. Designated diagnosis – light morphologic features plus two or more supplemental positive results to stains for adenosine triphostphatase, s-100 protein, a-D-mannosidase, and peanut lectin
3. Definitive diagnosis – light morphologic characteristics plus Birbeck granules in the lesional cell visicble with electron microscopy and/or positive results on staining for CD1a antigen on the lesional cell

Front 336

DIAGNOSTIC CRITERIA FOR SYSTEMIC MASTOCYTOSIS

Back 336

(Major and One Minor or 3 Minor criteria are needed)

MAJOR
1. Multifocal dense infiltrates of mast cells in bone marrow and/or other extra-cutaneous organs
MINOR
1. More than 25% of the mast cells in bone marrow aspirate smears or tissue biopsy sections are spindle shaped or display atypical morphology
2. Detection of a codon 816 c-kit point-mutation in blood, bone marrow, or lesional tissue
3. Mast cells in bone marrow, blood, or other lesional tissue expressing CD25 or CD2
4. Baseline total tryptase level of greater than 20 ng/mL

Front 337

BEHCETS CRITERIA – 2004 JAPAN

Back 337

MAIN POINTS
1. MAIN SYMPTOMS
a.Recurrent oral aphthous ulcers
b.Skin lesions:i.Erythema nodosum ii.Superficial thrombophlebitis iii. Papules; skin hypersensitivity
c.Ocular lesions d.Iridocyclitis or sequelae
e.Posterior uveitis or sequelae
2.Genital ulcers
3.Additional symptoms
a.Arthritis without deformity or sclerosis b.Epidiymitis
c.Gastrointestinal lesions represented by ileocecal ulcerations
d.Vascular lesions
e.Central nervous system lesions moderate or severe

Front 338

BEHCETS CRITERIA – 2004 JAPAN
…Continued…

Back 338

4.Criteria for Diagnosis of disease types
a.Complete types: 4 main symptoms
b.Incomplete types: 3 main symptoms or 2 main symptoms and 2 additional sx or typical ocular lesions and another main symptom or 2 additional sx
c.Suspected disease: typical main symptoms not fulfilling the criteria for an incomplete type
d.Special lesions: certain gastrointestinal, vascular, and nervous system lesions.
5.Clinical lab data contributing to the diagnosis (not essential)
a.Negative or positive pathergy test
b.Negative or positive prick test to vaccinate for streptococci
c.Inflammatory response
d.+ HLA-B51
e.Other pathologic findings
6.Additional points
a.Non-typical symptoms should not be diagnosed as Behcet’s disease
b.One of the criteria (a) to (c) of the skin lesions and (a) or (b) of the ocular lesions is enough for the diagnosis of the relevant symptom if the lesion occurs frequently

Front 339

REVISED INTERNATIONAL CRITERIA FOR BEHCET’S DISEASE

Back 339

Diagnosis of Behcet’s disease is made with a score of 3 points:
1 point = oral aphthosis
1 point = skin manifestations (pseudo-folliculitis, skin aphthosis)
1 point = Vascular lesions (phlebitis, superficial phlebitis, large vein thrombosis, aneurysm, arterial thrombosis)
1 point = Positive pathergy test
2 points = genital aphthosis
2 points = Ocular lesions

Front 340

CRITERIA FOR PRIMARY RAYNAUD PHENOMENON – Allen and Brown and LeRoy and Medsger

Back 340

1. Vasospastic attacks precipitated by exposure to cold or emotional stimuli
2. Bilateral involvement of extremities
3. Normal vascular examination with symmetric peripheral pulses and normal nail fold capillary microscopy
4. Absence of gangrene or, if present, limited to the skin of the fingertips
5. No evidence of an underlying disease, drug, or occupational exposure that could be responsible for vasospastic attacks
6. Negative anti-nuclear antibody test
7. Normal ESR
8. History of symptoms for at least 2 years

Front 341

CENTERS FOR DISEASE CONTROL AND PREVENTION CASE DEFINITION OF STAPHYLOCOCCAL TOXIC SHOCK SYNDROME

Back 341

MAJOR CRITERIA (ALL 4 MUST BE MET)
1. Fever
a. Temperature >38.9
2. Rash
a. Diffuse macular erythema
3. Desquamation
a. 1-2 weeks after onset of illness, particularly on palms and soles
4. Hypotension
a. Systolic BP<95 mm Hg for adults, or less than fifth percentile by age for children < 16 yr of age, or orthostatic syncope


NORMAL TEST RESULTS (IF PERFORMED)
1. Blood, throat, or CSF cultures (blood cultures may be positive to S. Aureus)
2. No rise in titer in antibody tests for RMSF, leptospirosis, or measles

Front 342

CENTERS FOR DISEASE CONTROL AND PREVENTION CASE DEFINITION OF STAPHYLOCOCCAL TOXIC SHOCK SYNDROME

Back 342

MULTISYSTEM INVOLVEMENT (3 OR MORE MUST BE MET)
1. Gastrointestinal
a. Vomiting or diarrhea at onset of illness
2. Muscular
a. Severe myalgia or creatine kinase level twice upper limit of normal
3. Mucous membrane
a. Oropharyngeal, conjunctival, or vaginal hyperaemia
4. Renal
a. Blood urea nitrogen or creatininen level twice upper limit of normal, or >5 white blood cells per high power fields in urine in absence of urinary tract infection
5. Hepatic
a. Total bilirubin, aspartate aminotransferase, or alanine aminotransferase level at least twice upper limit of normal
6. Hematologic
a. Platelets <100000/mm3
7. CNS
a. Disorientation or alterations in consciousness without focal neurologic signs when fever and hypotension are absent

Front 343

MODIFIED DUKE CRITERIA FOR INFECTIVE ENDOCARDITIS

- Clinically definite case is defined as fulfilling 2 major, one major plus 3 minor, or 5 minor criteria
- Clinically suspicious case is defined as fulfilling one major and one minor, or 3 minor criteria

Back 343

MAJOR CRITERIA
1. Microbiologic
i. Two separate blood cultures positive for typical microorganisms
ii. Persistently positive blood culture for typical microorganism
iii. Single positive blood culture for Coxiella burnetii or a phase I immunoglobulin G antibody titer to Coxiella burnetii great than 1:800
2. Evidence of endocardial involvement
i. New valvular regurgitation
ii. Positive echocardiogram showing oscillating echogenic intracardia mass at the site of endocardial injury, a periannular abscess, or new dehiscence of a prosthetic valve

Front 344

MODIFIED DUKE CRITERIA FOR INFECTIVE ENDOCARDITIS
MINOR CRITERIA

Back 344

MINOR CRITERIA
1. Predisposition to infective endocarditis
2. Fever
3. Vascular phenomena such as Osler nodules or Roth spots
4. Immunologic factors such as a positive rheumatoid factor or glomerulonephritis
5. Serologic evidence of active infection not meeting microbiologic major criteria

Front 345

DIAGNOSIS OF LEPROSY

Back 345

1 of 2 criteria
1. A consistent peripheral nerve abnormality or
2. the demonstration of acid-fast bacilli in tissues

Front 346

CRITERIA FOR THE USE OF INVESTIGATIONAL VARICELLA-ZOSTER IMMUNE GLOBULIN FOR THE PROPHYLAXIS OF VARICELLA

Back 346

1. immunocompromised patients
2. neonates whose mothers have signs and symptoms of varicella around the time of delivery (i.e. 5 days before to 2 days after)
3. premature infants born at >=28 weeks of gestation who are exposed during the neonatal period and whose mothers do not have evidence of immunity
4. premature infants born at <28 weeks of gestation or who weigh < 1000 grams at birth and were exposed during the neonatal period, regardless of maternal history of varicella disease, or vaccination.

5. Pregnant women.