Peds Board Review - Derm

Front 1

Prodromal symptoms: fever, malaise, dry (croupy) cough, coryza, and conjunctivitis with clear discharge and marked photophobia
Pathognomonic enanthem

Back 1

(Koplik spots)
FIRST
Rubeola, Measles

Hint 1

Exanthem is seen first on day 3 or 4, as the prodromal symptoms and fever peak in severity
Blotchy, erythematous, blanching, maculopapular eruption that appears at the hairline and spreads cephalocaudally over 3 days, ultimately involving the palms and soles
Once generalized, the rash becomes confluent over proximal areas but remains discrete distally

Front 2

White/ red strawberry tongue
Flushed face, perioral pallor, and a diffuse, blanching, erythematous rash that has a sandpapery consistency on palpation. Palatal petequiae.
Pastia lines (accentuation of the rash in the skin flexures/ creases)
Desquamation occurs in fine, thin flakes as the acute phase of the illness resolves and is proportional to the intensity of the exanthem.

Back 2

SECOND
Scarlet Fever, Scarlatina

Hint 2

Tx within 7 days to prevent rheumatic fever

Streptococcus pyogenes

Front 3

The exanthem is a discrete, pinkish red, fine maculopapular eruption, which, like measles, typically begins on the face and spreads cephalocaudally. The rash becomes generalized within 24 hours, then begins to fade, clearing completely by 72 hours.
The occipital, posterior cervical, and postauricular LAD
Arthritis and arthralgias are frequent in young girls

Back 3

THIRD: Rubella, German measles

Hint 3

Forchheimer spots are seen in some patients on day 1 of the rash
Little or no prodrome
Encephalitis is the most serious complication

Front 4

Onset is heralded by fever and irritability, often accompanied by vomiting followed by the development of diffuse erythroderma that spreads rapidly from head to toe and simulates the appearance of a sunburn
In contrast to streptococcal scarlet fever, the involved skin is tender, even to light touch.
Within 1 to 3 days, thin-walled, flaccid, bullous lesions appear and rupture soon after formation followed by epidermal separation

Back 4

Staphylococcal Scalded Skin Syndrome (SSSS), Ritter's disease

Hint 4

Nikolsky sign (separation of the skin in response to stroking)

The primary infection is usually mild, with purulent nasopharyngitis, conjunctivitis, impetigo, and infections of the umbilicus and circumcision sites seen most commonly.

Staphylococcus aureus’
phage group II coagulase-positive’s epidermolytic exotoxin

Front 5

PRODROME: low-grade fever, malaise, myalgias, and vomiting.
DISEASE: abrupt increase in fever with chills, worsening myalgias, repetitive vomiting, abdominal pain, severe watery diarrhea, orthostatic dizziness, and weakness
RASH: diffuse erythroderma, mimicking sunburn.
SHOCK: Hypotension and oliguria may become prominent, accompanied by alterations in level of consciousness.

Back 5

FOURTH: Toxic Shock Syndrome

Hint 5

Menstruating women (tampon) or skin lesion, an abscess, or purulent conjunctivitis as the primary focus of infection.
Desquamation usually beginning a week after onset of the rash. It is most prominent over the palms and soles and in the periungual areas, and the skin is shed in thick casts.
coag-positive staphylococci of phage group I or III

Front 6

Thin walled large flaccid bullae.

Back 6

Bullous impetigo

Hint 6

S. aureus

Front 7

Slapped check
Lacy, reticulated erythema on the extremities or trunk.
Papular purpuric “gloves and socks” syndrome

Back 7

FIFTH: Erythema Infectiosum

Hint 7

Parvo B 19

After 3 to 5 days, the eruption fades, although it may return for up to 4 months following exercise, overheating, or sun exposure.

Front 8

Exanthem of this disorder usually appears abruptly after 3 days of high fever and irritability. Fever stops.

Back 8

SIXTH: Exanthem subitum, Roseola infantum

Hint 8

6 and 36 months with high fever (>39C) without a source

Human Herpes Virus 6

Front 9

Non-purulent conjunctivitis, rhinitis, pharyngitis with or without exudate, and a discrete, blanching, maculopapular rash

Back 9

Adenovirus

Front 10

Enanthem: mildly painful, shallow, yellow ulcers surrounded by red halos in the oral mucosa (it is called herpangina when the rash is not present)
Exanthem: thick-walled, gray vesicles on an erythematous base involves the palmar, plantar, and interdigital surfaces of the hands and feet and sometimes the buttocks.

Back 10

Hand-foot-mouth

Hint 10

Coxsackievirus
A16

Front 11

- Non specific maculopapular rash
- Eyelid edema is found in 50%
- Severe pharyngotonsillitis
- Generalized lymphadenopathy epitrochlear
- Gianotti-Crosti syndrome

Back 11

Infectious mononucleosis
EBV

Hint 11

Up to 80% of patients with infectious mononucleosis experience "ampicillin rash" if treated with ampicillin or amoxicillin

Front 12

Multistage (papules, vesicles, and umbilicated and scabbed lesions)

Back 12

Varicella: chickenpox
varicella-zoster virus

Hint 12

GAS super infection
VZIG within 96 hours of exposure for immunocompromised children who have not had varicella previously (including those on short-course, high-dose steroids)

Front 13

Rash and fever.
Waterhouse-Friderichsen syndrome (shock and purpura) = adrenal hemorrhage and secondary adrenal insufficiency.
50% have NL WBC (overwhelmed immune system)
Only about 20% of these patients have meningitis.
Deaths within 24 hours circulatory collapse and CHF caused by endotoxic shock and myocarditis

Back 13

Meningococcemia
Neisseria meningitides
gram-negative diplococci.

Hint 13

Antimicrobial prophylaxis with RIFAMPIN, Ceftriaxone or cipro for members of the same household or persons in "closed communities" like military barracks, dormitories, or day-care centers.

Front 14

Transmission occurs when a person is bitten by an infected Ixodes species tick (deer tick).
Ixodes scapularis (formerly Ixodes dammini) is the most common vector.
RASH: erythema migrans (50% of cases) begins as a red papule or macule at the site of the tick bite and often goes unrecognized. The lesion gradually enlarges (to a median size of 15 cm), forming a large plaque, which tends to clear centrally, giving it an annular configuration.
Systemic symptoms: CNS: aseptic meningitis, VII paralysis, optic neuritis. HEART: A-V block or myopericarditis.
The arthritis of Lyme disease is a late manifestation, seen in up to 50% of untreated patients: pauciarticular, involving large joints (knee).
Late neurologic manifestations include encephalitis, encephalopathy, ataxia, radiculoneuritis, and myelitis.

Back 14

Lyme disease
tick-borne infection caused by the spirochete Borrelia burgdorferi

Hint 14

TREATMENT
> 8 DOXYCYCLINE for 14-21d.
< 8 AMOX or CEFUROXIME

DISSEMINATED OR LATE DISEASE

21-28 days.




NE of the USA (Connecticut)

Front 15

Usually transmitted to humans by the bite of an infected tick.
Mortality is as high as 5% to 7%.
Splenomegaly (30% to 50%).
The rash begins distally on the wrists, ankles, palms, and soles, usually appearing as an erythematous, blanching, fine, macular or maculopapular eruption. It then spreads centripetally and becomes petechial.

Back 15

Rocky Mountain Spotted Fever

Rickettsia rickettsii
obligate intracellular parasites

Hint 15

Thrombocytopenia and NL/ low WBC and hyponatremia.
TREATMENT
Doxycycline (regardless of age)

Front 16

1. A vascular malformation or port-wine stain over the face that involves the cutaneous distribution of the ophthalmic division of the V nerve
2. Ipsilateral leptomeningeal angiomatosis with associated intracranial calcifications
3. A high incidence of MR and ipsilateral ocular complications like buphthalmos (corneal enlargement), glaucoma or a coloboma
Seizures and a facial port-wine stain = ______ - _____ syndrome, which can be confirmed by CT scan. CT buzz words: progressive ipsilateral cerebral atrophy, serpiginous calcifications of brain parenchyma and intracranial calcifications

Back 16

Sturge-Weber

Hint 16

Only patients with lesions involving the forehead and upper eyelid are at risk for associated neuro-ocular complications
Repeated ophthalmologic and CT examinations are indicated only in this high-risk group, which has a 10% to 20% incidence of associated intracranial angiomas.

Front 17

The characteristic skin lesion of tuberous sclerosis are
Ash-leaf spots: congenital, well demarcated, 1- to 3-cm macular hypopigmented lesions on the trunk.
Angiofibroma (adenoma sebaceum): erythematous papules over the nose and malar region of the face. ~40% of children with TS demonstrate these lesions by 3 years of age.
Shagreen patch, a plaque of thickened skin with a cobblestone or orange-peel texture often seen on the dorsal aspect of the trunk.
Periungual fibromas
Oral examination may reveal pitting of dental enamel

Back 17

Tuberous sclerosis
A.D.
Wood's light exam: any child, with a fair complexion + idiopathic seizures in infancy.

Up to 0.5% of normal newborns an isolated hypopigmented macule is a NL variant (nevus depigmentosus)

Hint 17

Seizures (96%)
Mental retardation (60%)
Intracranial calcification (49%)

Tumors of various organs (cardiac rhabdomyoma (66%), renal angiomyolipomas (75%), pulmonary lymphangiomyomatosis (<2%), and hepatic hamartoma)

Front 18

Port-wine stain that is usually located over the lateral aspect of one leg
Hypertrophy (usually hemihypertrophy) and lymphedema

Back 18

Klippel-Trénaunay syndrome

Front 19

Germ line mutations in the VHL tumor suppressor gene (3p25-26).
Multiorgans, including the cerebellum(hemangioblastomas), spinal cord, medulla, retina (angiomata), kidney, pancreas, and epididymis.

Back 19

von Hippel-Lindau disease
AD

Hint 19

Renal carcinoma is the most common cause of death

Front 20

Multisystem, degenerative disorder characterized by ataxia, oculocutaneous telangiectasia, immunodeficiency, and a high incidence of neoplasia (ALL or lymphoma).
Recurrent sinus and pulmonary infections due to cellular and humoral (IgA and IgM deficiency).

Back 20

Ataxia-Telangiesctasia
AR

Hint 20

Tremors of the head may be seen before 1 yr, and ataxia is evident when the child first walks.
Ocular telangiectasia at 6yo.

Front 21

Present at birth, manifest as a yellowish-tan, waxy linear lesion
Nevus may be found on the scalp, face, neck, trunk, or extremities
15% to 20% risk of malignant degeneration
Association with seizures and mental retardation has been reported.

Back 21

Linear nevus sebaceus of Jadassohn

Hint 21

The risk of neurologic abnormalities is greatest when the cutaneous lesion is located in the midfacial area.

Front 22

Congenital neurocutaneous disorder in which an epidermal nevus (raised, often warty hyperpigmented lesions typically in a linear pattern) is seen in association with neurologic dysfunction (seizure, MR, DD).

Back 22

Epidermal nevus syndrome

Hint 22

Unilateral hemimegalencephaly with intractable seizures is the commonest CNS abnormality

Front 23

Cutaneous, neurologic, ophthalmologic, and dental manifestations
1st stage: Linearly distributed vesicles on an erythematous base
2nd stage: lesions evolve into warty papules, which can have thick overlying crusts
3rd stage: swirled hyperpigmented patches replaced the warty lesions by 8 months of age

Back 23

Incontinentia pigmenti

X-linked dominant syndrome

Hint 23

Neurologic and ophthalmologic occur in about 30% of patients: seizures, CVA, developmental delay, mental retardation, and microcephaly.

DDX: neonatal herpes