Flashcards in Peds Derm Deck (85)
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1
Name the typical triad of Hand-Schiller-Christian Disease
Diabetes insipidus
Bone lesions *osteolytic bone lesions, cranium
Exopthalmos
This is a LCH, onset between 2-6
2
Acute disseminated form of LCH, presents before age 2
Letterer-Siwe Disease
3
LCH presenting in older children, a localized form of LCH
-asymptomatic granulomatous lesions involving the bone (cranium), spontaneous fractures
Eosinophilic Granuloma
4
What is another name for congenital self healing reticulohisticytosis, a self limited version of LCH limited to the skin
Hashimoto-Pritzker disease
5
Hypertrichosis and hyperpigmentation on the medial thighs
-associated with low height, hyperpigmentation, hearing loss, heart anomalies, hearing loss, hyperglycemia
H syndrome (defect in SLC29A3), encodes nucleoside transported
6
What do you manage the DI of Hand-Schiller-Christian disease with?
Vasopressin
(Remember this is a LCH with a triad of osteolytic bone lesions, diabetes insipidus, and exopthalmos)
7
Most common location for JXG
Head and neck
8
What percentage of JXG appear within the 1st year of life?
75%
9
What two diseases is JXG associated with?
NF and JMML
10
What other specialty do you want to send a kid to if they have multiple JXGs?
Optho
11
Risk factors for ocular JXG
>2 years old or multiple JXGs
12
Numerous red/brown macules and papules on the face in an infant
Self limited
No internal involvement
Looks like JXG but without Touton giant cells
Benign cephalic histiocytosis
13
What is the adult form of benign cephalic histiocytosis?
Generalized Eruptive Histiocytosis (both likely JXG variants)
14
How can you tell the difference between Sclerema Neonatorum and Subcutaneous Fat Necrosis of the Newborn on pathology?
Sclerema Neonatorum has LESS inflammation. Subq fat of the newborn is a localized version of Sclerema neonatorum, both are panniculitis, and present as woodyness of the skin. Subq fat of the newborn will have an intense inflammatory infiltrate on path
15
What type of infiltrate do each of these subcorneal pustules contain?
Transient neonatal pustular melanosis
Erythema Toxicum Neonatorum
Neonatal Cephalic Pustulosis
Transient neonatal pustular melanosis - neuts
E tox - eos
Neonatal cephalic pustulosis - neuts
16
What drug in hypothyroid moms can cause aplasia cutis in the baby?
Methimazole
17
What syndrome has aplasia cutis of the scalp with skull defects, CMTC, and limb defects?
Adams-Oliver-Syndrome (think abt the Adams family - creepy girl with a wide part, cmtc and short limb)
18
Bilateral temporal aplasia cutis, abnormal eyelashes, leonine facies, upward slanting eyebrows, Peurto rican
Seitles Syndrome *setters syndrome - like a volleyball setter
Defect in the TWIST2 gene
19
Dystrophic EB + aplasia cutis
Bart Syndrome
20
Another name for Trisomy 13
Pattau Syndrome
21
Most common eye finding associated with CMTC
Glaucoma
22
Leading infectious cause of deafness and mental retardation in newborns
CMV
23
Congenital HSV infections characteristically harbored in what part of the brain
Temporal lobes
24
Which type of HSV is most common in congenital HSV?
What percentage of these infections are aquired perinatally?
HSV2
85%
25
What is the mortality rate of untreated congenital HSV infections?
50-75%
26
Name three congenital infections that give you blueberry muffin baby
CMV
Rubella
Toxoplasmosis
27
Which congenital infection gives you hepatosplenomegaly?
Rubella
28
What is Higoumenakis sign?
Congenital thickening of the medial aspect of the clavicle in LATE congenital syphilis
29
Which diaper dermatitis is essentially acrodermatitis enteropathica with seizures?
Multiple carboxylase deficiency (deficiency in holocarboxylase)
30
