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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

What percentage of psoriasis presents before age 15?

25%