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76 Cards in this Set
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Mucocutaneous lymph node syndrome
Exanthem: non-specific macular or fine papules, perianal desquamation early Oral signs: red, fissured lips, “strawberry” tongue, diffuse oropharyngeal erythema Hand and foot lesions: indurated erythema of hands and feet, desquamation of digits 2 weeks after onset |
Kawasaki Disease
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Fever: >38.3o C > 5d
Cervical lymph node enlargement Bilateral conjunctival injection Cardiac abnormalities are at high risk, need to send to cardiology Fever and 4/5 of other criteria must be present Affects children <5 years old Males>Females Etiology: Probably infectious in origin Perianal infection |
Kawasaki Disease
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No specific laboratory test exists
A mild-to-moderate normochromic anemia is observed in the acute stage Moderate to alarmingly elevated WBC count with a left shift.(day 1-11) ESR, CRP level, and serum alpha1-antitrypsin level are elevated. Culture results are all negative. Subacute stage, platelet count elevation is the outstanding marker.(day 11-21) It begins to rise in the second week and continues to rise during the third week. Levels as high as 2 million have been observed. The acute reactive markers remain elevated. Convalescent stage (day 21-60), the levels of platelets and other markers begin to return to normal. |
Kawasaki Disease
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Imaging Studies:
An echocardiogram (ECHO) is the study of choice to demonstrate coronary artery aneurysms. During the acute stage, a baseline ECHO is important. The ECHO should be repeated in the 2nd or 3rd week and 1 month after laboratory results have normalized. Repeat ECHO at 1-year, even when the first ones show no aneurysm. If the ECHO results are abnormal at any point, the child should be referred to a pediatric cardiologist for a complete cardiac workup and follow-up care. An ultrasound of the gallbladder may be necessary if any suggestion of liver or gallbladder dysfunction is present. A chest radiograph should be obtained to assess baseline findings and to confirm clinical suspicion of congestive heart failure. |
Kawasaki Disease
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Other Tests:
An electrocardiogram (ECG) indicates the presence of various conduction abnormalities. A select group may require cardiac catheterization. Coronary artery bypass grafting and cardiac transplantation have been needed. Treatment: IV gamma globulin Aspirin Dipyridamole (Persnatine) |
Kawasaki Disease
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Clinical features :
Exanthem on day 4 of fever: confluent maculopapular rash starts on forehead spreads to toes in 3 days or villiform (maculopapular rash of measles) Spreads down and out and will still be one the forehead when |
Measles:
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Enanthem: Koplik’s spots around day 2 of fever,
Coryza, Cough, Conjunctivitis Photosensitivity prodrome Etiology: Measles virus Can be fatal |
Measles:
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Clinical features:
Exanthem on day 1 of fever: starts on face and spreads in 24 hours As it spreads, it will fade from the face |
Rubella:
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Exanthem: Forchheimers’ sign: palatal petechiae on roof of mouth. Young adults with flu-like illness prodrome
“3 day” Not dangerous with exception of pregnant women: can get "blueberry baby" |
Rubella:
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"German Measles"
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Rubella:
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Acute inflammatory/immunologic reaction caused by a number of etiologies
Clinical findings: Painful, tender nodules on the lower legs, fever, malaise, 50% with arthralgias Very painful, almost always on lower legs Gender: Three times more common in females than males |
Erythema Nodosum
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Etiology: many causes:
Infection: Group A ß-hemolytic Strep and others Drugs: Sulfa, OCP Others: Sarcoidosis, Ulcerative colitis, Behçet’s (Sarcoidosis: get CXR, black female) (Behcet's: chronic condition caused by disturbances in immune system, Common symptoms include: mouth ulcers, genital ulcers, eye inflammation and arthritis) Idiopathic in 40% Treatment: Spontaneous resolution in 6 weeks depending on etiology Bed rest, Symptomatic treatment |
Erythema Nodosum
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“Palpable purpura” are the classic lesion; urticarial lesions are the next most common (looks just like hives, if it moves it is hives, if it is stationary it may be vasculitis),
-negative diascopy -Petichiei all over |
Vasculitis
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Occasional fever and malaise
Review of systems: complete ROS; look for neuritis, abdominal pain, arthralgia, hematuria Etiology: Deposition of immune complexes in tissue Idiopathic in 50% Associated with: Drugs (lasix -furosemide) Infection Connective tissue diseases (usually Lupus, Rheumatology is closely related) Neoplasm |
Vasculitis
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Many things can cause this and difficult to determine cause
Bottom line: do initial work up and if unsure, consult Rheumatology Laboratory: ESR CBC UA BUN/Cr LFTs Other laboratories ANA/NAP/RF cANCA/pANCA Cryoglobulins Hepatitis panel/HIV CRP Blood cultures RPR/VDRL Skin biopsy Angiography Treatment: Identification of underlying disease |
Vasculitis
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Clinical features: Fever, heart murmur and:
1) Janeway lesions: red or hemorrhagic macules on the palms and soles (DDx: gonorrhea) 2) Osler’s nodes: tender, purplish, subcutaneous nodules on digits and palms 3) Subungual splinter hemorrhages: in the middle one-third of the nail (if distal, more likely to be from trauma) Acute bacterial endocarditis: (1, 2) - result from septic embolism Subacute bacterial endocarditis: (2, 3) - from circulating immune complexes |
endocarditis
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Clinical features: Fever, chills, weakness, HA precede the rash
Pink-red macules on wrists, ankles and forearms - appear on day 4 of fever (range 2-6 days) Macules spread to palms/soles, then centrally, petechiae seen within the rash 2-4 days later |
Rocky Mountain Spotted Fever
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+ Rumpel-Leede test = sphygmomanometer pressure induces petechiae
Petechiae coalesce to become hemorrhagic and gangrenous lesions |
Rocky Mountain Spotted Fever
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Etiology: Rickettsia rickettsii transmitted via wood tick or dog tick (name starts w/ dermatocenter)
Diffuse vasculitis secondary to rickettsial invasion of capillaries, arterioles and venules Treatment: doxycycline or chloromaphenoil |
Rocky Mountain Spotted Fever
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what is: Rocky Mountain “spotless” fever
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systemic illness without the rash
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Clinical features: “Dermatitis-arthritis” complex
Grown on thayer martin plate Characterized by painful, red vesiculopustules Occasionally hemorrhagic macules 2mm-2cm Seen on palms, soles, over joints Fever, chills, malaise accompany eruption Unilateral painful joint Get cultures on any discharge Pelvic exam Most common in women with asymptomatic anogenital disease Polyarthralgia of the distal extremity joints: knees, elbows, ankles, wrists Myalgias or tenosynovitis |
Gonococcemia
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Etiology: Dissemination of Neisseria gonorrhea
Gonococci may be found in blood, skin lesions, joints and GU tract |
Gonococcemia
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Clinical features:
Petechiae Irregularly shaped with central gray vesicle on trunk and extremities Maculo-papular rash transiently resembles viral exanthem Variable presentation: suspect if fever, petechiae, sx of meningitis Petechiae coalesce to form ecchymotic areas Centrally hemorrhagic bullae necrose Dx: fever, petechia, sx of meningitis DDx: RMSF |
Meningococcemia
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Etiology: Neisseria
Direct involvement of cutaneous vessels by bacteria or via endotoxin Cause local endothelial damage, with thrombosis and infarction of overlying skin Labs: blood cultures, CBC, plt count, BUN, CrCl, coag series, LP Form of vasculitis but secondary to infection Present as sick pt and acts quick and workup is done emergently |
Meningococcemia
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Clinical features: Red, confluent macular “butterfly” eruption in the malar area
Has rash on hands, but spares the knuckles Systemic or discoid (skin) |
Systemic Lupus Erythematosus
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what % of discoid lupus will progress to systemic lupus
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10%
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Discoid will only show discoid plaques, butterfly is only with systemic
Seal has discoid lupus Discoid plaques, Palpable purpura, Photosensitivity, Alopecia, Mucosal ulcers Systemic involvement: fatigue (out of the ordinary), fever, weight loss, arthralgia, arthritis in systemic, the most common derm presentation is discoid plaques Renal, cardiac, pulmonary, CNS |
Systemic Lupus Erythematosus
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Epidemiology: M:F is 1:8, Onset 3rd and 4th decades
Etiology: Immune complexes at DE (dermal-epidermal) junction Laboratory: +ANA; DS-DNA, +DIF ANA is sensitive but not specific DS: double strand Treatment: Antimalarials, Prednisone, Immunosuppressives |
Systemic Lupus Erythematosus
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American College of Rheumatology 11 criteria:
Malar rash (rash on cheeks). Discoid lupus (red, scaly patches on skin which cause scarring) Photosensitivity (exposure to ultraviolet light causes rash). Oral ulcers: include oral or nasopharyngeal ulcers Arthritis: nonerosive arthritis of two or more peripheral joints, with tenderness, swelling or effusion Renal disorder: More than 0.5 g per day protein in urine, or cellular casts seen in urine under a microscope. Neurologic disorder: Seizures or psychosis. Serositis: Pleuritis (inflammation around the lungs) or pericarditis (inflammation around the heart) Hematologic disorder: Hemolytic anemia (low RBC count) or leukopenia (white blood cell count<4000/ul), lymphopenia ( <1500/ul ) or thrombocytopenia (<100000/uL) in the absence of offending drug. Anti-nuclear antibody test positive. Immunologic disorder: Positive anti-Sm, anti-ds DNA, anti-phospholipid antibody and/or false positive serological test for syphilis |
Systemic Lupus Erythematosus
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Clinical features: Hands/feet - Raynaud’s phenomenon
Edema Contractures Sclerodactyly (flexion contractures in fingers) Ulcers Telangiectasias in periungual area (dilated capillary loops) Face/trunk – edema perioral furrowing and eventual fibrosis (will initially notice that skin is shiny and taut) |
scleroderma
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Variant - CREST syndrome
C=calcinosis cutis (calcium deposits in skin that are palpable) R=Raynaud’s E=esophageal dysmotility (reflux) S=sclerodactyly T=telangiectasias |
scleroderma
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Review of systems
Stiffness Arthritis Dysphagia Dyspnea Epidemiology: M:F 1:4, 30-50 years Etiology: Unknown Treatment: Symptomatic immunosuppresives |
scleroderma
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Clinical features: Periorbital violaceous “heliotrope” macular eruption with edema
Gottron’s papules on the knuckles, Periungual telangiectasias |
Dermatomyositis
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Epidemiology: Juvenile; >40 years of age
(presentation: difficulties combing hair from shoulder girdle weakness, difficulty climbing stairs) Review of systems: progressive proximal muscle weakness dysphagia photosensitivity ? Association with internal malignancy >55 years Etiology: Inflammatory disease of dermis and striated muscle Diagnosis: Proximal muscle weakness with elevated CPK and aldelase Treatment: Prednisone |
Dermatomyositis
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Clinical features: “Apple-jelly” infiltrated plaques on the nose, cheeks, earlobes, arms
Skin lesions are asymptomatic, Shape is annular or polycyclic annular and indurated |
Sarcoidosis
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Review of systems: fever, fatique, dyspnea, uveitis
Radiographic studies: hilar lymphadenopathy, pulmonary infiltrates Epidemiology: M=F, More common in U.S. blacks, <40 years Etiology: Infiltrative granulomas of tissue Laboratory: ACE elevated, Hypercalcemia, Hypergammaglobulinemia Biopsy: DDx GA granular anularis? Treatment: Intralesional or topical steroids for skin disease, Systemic corticosteroids for ocular or active pulmonary disease |
Sarcoidosis
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Clinical features:
Acute - erythema chronica migrans Annular lesions at the site of tick bites Fever Chills Myalgia Weakness Headache Photophobia HSM Adenopathy |
Lyme Borreliosis
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Chronic -
acrodermatitis chronica atrophicans with dermal atrophy and fibrosis |
Lyme Borreliosis
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Carditis, arthritis, meningitis, encephalomyelitis
Etiology: Borrelia burgdorferi, transmitted via tick bite (deer tick - Ixodes species) Diagnosis: Acute - based on characteristic clinical findings Chronic - specific serologic tests Treatment: Regimen based on extent of involvement and stage of disease |
Lyme Borreliosis
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(ends in "s", present in sixties, sepsis, severe, superficial)
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Pemphigus Vulgaris
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Onset 50-60 years
Rarely pruritic Sometimes painful flaccid bullae, erosions oral lesions Positive Nikolsky's sign (twist skin or draw line on skin and push on blister to see if it moves) Majority of patients have painful mucosal membrane lesions treat as if they were a burn patient Treatment - oral glucocorticoids and other immunosuppresive agents |
Pemphigus Vulgaris
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Clinical features: urticaria-like lesions precede or occur simultaneously with bullous pemphigoid; Some are target-shaped
stationary Oral mucosal involved: 20% Pruritis is marked Etiology: Direct immunofluorescence on skin biopsy; IgG to bullous pemphigoid antigen in the subepidermal space |
Pre-bullous Bullous Pemphigoid
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Onset >60 years of age
negative Nikolsky's sign Pruritic skin lesions (very itchy) Oral mucous membrane lesions in 10-35% Self-limited in many cases Treatment with oral glucocorticoids alone or with azathioprine, also dapsone, tetracycline, niacinamide niacin and tetracycline work very well |
Bullous Pemphigoid
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Oral mucosal bullae/erosions and conjunctivae which scar
F:M 2:1 Symblepharon: fusion of conjunctiva to sclera Treatment glucocorticoids, dapsone, cyclophosphamide, azathioprine, tetracycline, niacinamide and topical steroids |
Cicatricial Pemphigoid
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Clinical features: Yellow-orange papules, plaques, nodules
Normolipoproteinemic or hyperlipidemic palpebrum usually normolipoproteinemic tendineum, palmar, tuberous, eruptive associated with elevated lipids Etiology: Collections of cells (lipid-laden macrophages) in the dermis Treatment: Diet and lipid lowering agents, if needed |
Xanthoma
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Von Recklinghausen’s
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Neurofibromatosis
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Clinical features: Cafe-au-lait macules - >5 lesions, diameter, >=0.5cm in kids <5 years of age
Axillary or inguinal freckling Neurofibromas or plexiform neuromas Rule of fives: under age of five |
Neurofibromatosis
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Other –
Lisch nodules Optic gliomas Acoustic neuromas Bowing of long bones Epidemiology: Autosomal dominant Etiology: Neural crest tumors and hamartomas Elephant man |
Neurofibromatosis
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Clinical features: Hyperpigmentation and thickening of the skin of the neck and in body folds; appears “velvety”
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Acanthosis nigricans
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most common in armpits and back of neck, sometimes on knuckles "knuckle pads"
Skin markings are accentuated Paraneoplastic AN can involve palms/soles and vermillion border of the lips Classification: Type 1 - hereditary, benign Type 2 - insulin resistant states Type 3 - Pseudo-AN, seen with obesity Type 4 - Drug-induced (nicotinic acid, oral contraceptives) Type 5 - Malignant - Paraneoplastic |
Acanthosis nigricans
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most common presentation: pruritis
Hypothyroid rough dry skin facial puffiness mucin skin deposits flat affect thick tongue yellow tint to skin hair coarse and brittle loss of eyebrows vitiligo associated with hyper or hypothyroidism |
Thyroid disease/Myxedema
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pretibial area thick
raised, rough, waxy plaques exaggerated hair follicles orange peel appearance |
Thyroid disease/Myxedema
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hypercortisolism
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Cushing's
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Skin atrophy
Poor wound healing Increase skin infections Brown-purple striae Acne Hirsutism Plethora Telangiectasias central obesity buffalo hump moon face |
Cushing's
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hypocortisolism
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Addison's
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Atrophic adrenal cortex
Idiopathic Surgical ablation TB Inadequate ACTH levels Dysfunction of the adrenal axis Iatrogenic administration of po corticosteroids or overuse of topical steroids Hyperpigmentation (particularly in skin creases) Loss of body hair esp axilla Vitiligo |
Addison's
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Onset generally prior to age 30
F:M = 2:1 Types: Localized Generalized Subcutaneous Perforating Arcuate dermal erythema Actinic granuloma "kissing lesions" |
Granuloma Annulare
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Lesions persist and then resolve with time
Morphology Annular plaque Skin colored, erythematous to violaceous Single or multiple indurated papules develop over wks. or mos. to annular configuration Assoc with diabetes, rheumatoid arthritis Treatment: Class I topical steroid with or without occlusion PUVA |
Granuloma Annulare
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Occurs in 0.3% of diabetic patients
looks identical to GA under microscope clinically: they are nothing alike Only found in diabetics F:M = 3:1 Anterior and lateral surface of lower legs Small, dusky-red, elevated nodule, slowly enlarges, becoming a plaque with irregular outline, flattened or depressed as the dermis becomes atrophic Brownish-yellow with telangiectasias throughout Treatment not satisfactory : topical glucocorticoids under occlusion, intralesional steroids |
Necrobiosis Lipoidica Diabeticorum NLD
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Half and half nails: chronic renal failure
Pruritis: uremia, hyperparathyroidism, post-dialysis (hyper-phosphatemia) (dry skin accounts for 85% of pruritis)(also, hypo or hyperthyroidism) (anti-histamines: knocks person out) (narcotic antagonist) |
Cutaneous Manifestations of Renal Disease
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Pseudoporphyria: in dialysis patients, similar presentation to PCT
(phototoxic type reaction, vessicle on hand and dorsum of face that form millia, have hypertrochosis - excess facial hair where it shouldn't be) (can be caused by drug reaction) (real PCT: urine will flouresce. Pseudoporphyria: urine does not flouresce) |
Cutaneous Manifestations of Renal Disease
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Cutaneous Manifestations of Renal Disease
perforating folliculitis, seen in chronic renal failure with diabetes (sprouting horns) |
Kyrle's disease
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Cutaneous Manifestations of Renal Disease
calcium deposits in large vessels cause infarcts, large deposits of calcium (rock hard lesion with necrosis on top) |
calciphylaxix
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Post-transplant: increased risk of SCCa
(combo: sun exposure and immunosuppressed) (accutane: can slow conversion of keratinic areas to SCC) (Aldara and Effudex) |
Cutaneous Manifestations of Renal Disease
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Acute febrile neutrophilic dermatosis:
20% assoc with malignancy most commonly AML, drugs 30-50 years of age Female predominance (if seen, think leukemia)(be suspicious with anyone who has had bone marrow transplant) Fever, plaque forming inflammatory papules, peripheral leukocytosis and diffuse dermal neutrophilic infiltrate (topographical appearance) Red to bluish papules or nodules Plaques look like a mountain range relief Treatment – systemic glucocorticoids, colchicine |
Sweet’s Syndrome
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Predominantly seen in Allogeneic BMT, less commonly Autologous BMT, PSCT and Liver transplant
(BMT: bone marrow transplant)(PSCT: peripheral stem cell transplant) (allogenic: the guest tissue attacks the host) (different phases) Acute phase: faint erythematous macules often on palms, soles and pinnae (about 10-30 days post-transplant) (texture of oatmeal) Pruritis is uncommon Can progress to erythroderma, bulla, lichenoid Additional findings: elevated total bilirubin, diarrhea Differential: drug eruption, viral exanthem, TEN (toxic epidermal necrolysis: fatal drug reaction) Chronic phase: lichenoid and sclerodermoid types (about 60 days post- transplant) Treatment: Acute: Prednisone, FK-506, Anti-thymocyte globulin, monoclonal antibodies; Chronic: Prednisone, cyclosporine, azathioprine, lichenoid – PUVA; sclerodermatous – photophoresis, targretin |
Graft Versus Host Disease
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Multiple disorders based on enzyme defects in _________ metabolism (heme synthesis)
Most common ___________ with dermatologic manifestations: ________ cutanea tarda (most common), Variegate ________, Erythropoietic proto________ (next most common) Rare – Erythropoietic _________ (?vampire myth) Heme Pathway |
porphyria
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EP: autosomal recessive
UROGEN III cosynthase deficiency – presents in first few months of life, photosensitivity, mutilating skin lesions, hirsutism, erythrodontia PCT: variable, autosomal dominant assoc with Hepatitis C UROGEN decarboxylase, inherited or acquired, vesicles and bulla in dorsa of hands, scarring and small milia, hypertrichosis, mottled pigmentation, begin in 4th-5th decade (most common) tx: phlebotomy VP: autosomal dominant PROTOGEN oxidase – skin manifestations of PCT (but begin 2nd-3rd decade) and findings of AIP (abdominal pain, neuro and psychiatric manifestations) EPP: autosomal dominant (second most common) ferrochelatase deficiency – begins in early life, cutaneous photosensitivity with burning, stinging, pruritis becoming atrophic, waxy and pitted scars, premature aging |
The Porphyrias
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Ichthyosiform Disorders
autosomal dominant (very common) onset infance/childhood – hyperlinear palms, ichthyosis worse on lower extremities, keratosis pilaris, atopy triad: ichthyosis, keratosis, |
Ichythyosis vulgaris:
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Ichthyosiform Disorders
X-linked recessive onset birth to infancy – comma-shapred corneal opacities, cryptorchidism, hyperkeratosis very taut skin, don't sweat well |
X-linked ichthyosis
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Ichthyosiform Disorders
autosomal recessive onset birth often collodion presentation – ectropion, alopecia, heat intolerance |
Lamellar ichthyosis
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Ichthyosiform Disorders
(congenital bullous ichthyosiform erythroderma): autosomal dominant onset birth – verrucous, firm hyperkeratotic spines, blisters, erythroderma, palmoplantar keratoderma, frequent skin infections, pungent odor |
Epidermalytic hyperkeratosis
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Ichthyosiform Disorders
assoc with lamellar ichthyosis, congenital ichthyosiform erythroderma, Netherton’s syndrome and self-healing collodion baby |
Collodion baby
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Ichthyosiform Disorders
****seen in HIV infection, most common associations: sarcoidosis, autoimmune disease, Hodgkin’s lymphoma and drugs (lipid lowering agents and butyrophenone) (paraneoplastic sign) |
Acquired ichthyosis***
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a chronic skin condition that causes loss of pigment, resulting in irregular pale patches of skin.
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vitiligo
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a rare, multi-system genetic disease that causes benign tumours to grow in the brain and on other vital organs such as the kidney, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioural problems, skin abnormalities, lung and kidney disease
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tuberous sclerosis
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