Peds Derm Flashcards by Rachel Pflederer

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

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Acute disseminated form of LCH, presents before age 2

Letterer-Siwe Disease

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LCH presenting in older children, a localized form of LCH

-asymptomatic granulomatous lesions involving the bone (cranium), spontaneous fractures

Eosinophilic Granuloma

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What is another name for congenital self healing reticulohisticytosis, a self limited version of LCH limited to the skin

Hashimoto-Pritzker disease

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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

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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

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Most common location for JXG

Head and neck

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What percentage of JXG appear within the 1st year of life?

75%

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What two diseases is JXG associated with?

NF and JMML

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What other specialty do you want to send a kid to if they have multiple JXGs?

Optho

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Risk factors for ocular JXG

> 2 years old or multiple JXGs

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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

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What is the adult form of benign cephalic histiocytosis?

Generalized Eruptive Histiocytosis (both likely JXG variants)

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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

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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

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What drug in hypothyroid moms can cause aplasia cutis in the baby?

Methimazole

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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)

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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

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Dystrophic EB + aplasia cutis

Bart Syndrome

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Another name for Trisomy 13

Pattau Syndrome

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Most common eye finding associated with CMTC

Glaucoma

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Leading infectious cause of deafness and mental retardation in newborns

CMV

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Congenital HSV infections characteristically harbored in what part of the brain

Temporal lobes

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Which type of HSV is most common in congenital HSV?

What percentage of these infections are aquired perinatally?

HSV2

85%

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What is the mortality rate of untreated congenital HSV infections?

50-75%

Name three congenital infections that give you blueberry muffin baby

CMV Rubella Toxoplasmosis

Which congenital infection gives you hepatosplenomegaly?

Rubella

What is Higoumenakis sign?

Congenital thickening of the medial aspect of the clavicle in LATE congenital syphilis

Which diaper dermatitis is essentially acrodermatitis enteropathica with seizures?

Multiple carboxylase deficiency (deficiency in holocarboxylase)

What percentage of psoriasis presents before age 15?

25%

Treatment for PLEVA/PLC

Oral erythromycin

Most common type of juvenille PRP

Type IV Circumscribed juvenille form | *(type III is like classic PRP, but type IV is actually the most common)

Which type of juvenille PRP is like classic adult PRP?

Type III (Classic Juvenille form)

What does atypical juvenille PRP (Type IV) present like?

Like type III (classic PRP) but with scleroderma like changes as well

Lichen striatus will persist with what type of lesion months to years after the initial papular stage. This is usually the presentation clinically

Hypopigmentation

What does lichen striatus look like on pathology?

Lichenoid infiltrate around ECCRINE glands

KP atrophicans faciei/ulerythema ophyrogenes (which is the term for KP atrophicans of the lateral 1/3 of the eyebrows) occurs in what syndrome?

Noonan Syndrome

Ulerythema ophyrogenes

A/w Noonan syndrome. KP atrophicans of the lateral 1/3 of the eyebrow

Which have an increased number of melanocytes: lentigos or ephelides?

Lentigos. Ephelides have a normal number

Which do ephiledes have: | Increased melanocytes? Or increased melanosomes?

Increased melanosome, normal melanocytes (vs lentigenes which have an increased number of melanocytes)

What are the sizes for: Small congenital nevus Medium congenital nevus Large congenital nevus

Small: <1.5cm Medium: 1.5-10cm Large: 10-20 Giant: >20cm

Schimmelpenning syndrome

Epidermal nevus syndome. Basically epidermal nevi or ILVEN plus underlying CNS symptoms

PEODEN

Porokeratotic eccrine and osteodermal duct nevus - JGB2 mutation - kid with a large poor and keratotic spicules on the face - their children could have KID syndrome *so screen for oral SCC!!

What tumors can arise out of a nevus sebaceous?

Trichoblastoma SPEP BCC!

Nevus sebaceous has been linked to what gene?

PTCHD!

Multiple pilomatricomas may be associated with what disease? | And what cell protein is upregulated?

Myotonic Dystrophy | Upregulation of beta-catenin

What percent of plexiform NF can undergo malignant transformation?

2-13%

What percentage of children with urticaria pigmentosa get pulmonary symptoms?

0%! Trick questions! Children with UP do NOT get any pulmonary sxs. Kids with diffuse cutaneous mastocytosis do get pulmonary symptoms and also GI symptoms

Most common extracutaneous manifestation of systemic cutaneous mastocytosis?

Bone marrow >> GI. *boards question

Cellular markers for mastocytosis

CD34, CD117. (*CD117 so you can use imatinib to help these kids)

What can you give these kids who have GI symptoms from diffuse cutaneous mastocytosis?

Cromyln

Name three syndromes associated with lipomas

Gardner Bannayan Riley Rulvaca Syndrome MEN-1

Name two genoderms a/w aphasia cutis congenital

Adams-Oliver syndrome | Bart Syndrome

Teratogens a/w aplasia cutie

Methimazole

What kind of giant cells are found in JXG?

Touton giant cells

What percentage of JXGs have ocular involvement? And what kind of ocular involvement do you worry about?

0.5% can get Glaucoma or hyphens

What two stains are the Langerhans cells in LCH positive for?

S100+, CD1a+

Treatment for subcutaneous fat necrosis of the newborn

Screen for HYPERcalcemia for 6 months

How can you tell the difference on path between Sclerema neonatorum vs subcutaneous fat necrosis of the newborn?

Sclerema Neonatorum lack granulomatous inflammation

What type of laser would you treat a nevus of ota with?

Q switched laser - Ruby, Alexandrite, or ND-YAG

What branchial derivative is the tragus from? (Brachial arch vs cleft)

FIRST Branchial arch

Name the findings of PELVIS syndrome

``` Perineal hemangioma External genitalia malformation Lipomeningocele Vesicorenal abnormalities Imperforate anus Skin tag ```

Brown, orange, crusted plaques with vesicles and bulla in the perianal and perioral areas

Acrodermatitis enteropathica (zinc deficiency) *inherited form is when you wean off breast milk

What is the AR neonatal form of holocarboxylase synthetase?

Essentially inherited biotin deficiency! Presents pretty much like acrodermatitis enteropathica

Inherited form of Biotin deficiency

Holocarboxylase synthetase deficiency (AR)

A midline cervical cleft can contain what kind of ectopic tissue?

Salivary tissue

Three congenital infections which cause "blueberry muffin baby"

CMV Toxoplasmosis Rubella

Most common symptom of congenital rubella

Deafness (remember you also get blueberry muffin baby)

What trimester does getting this infection make blueberry muffin baby worse? (Rubella, toxo, CMV)

FIRST TRIMESTER

Early or late congenital syph: Saber shins

Late

Early or late congenital syph: Hutchison's teeth

Late

Early or late congenital syph: snuffles

Early

Early or late congenital syph: blueberry molars

Late

Early or late congenital syph: Higoumenakis sign

Late

Early or late congenital syph: Parrot's psuedoparalysis

Early

Early or late congenital syph: Parrots lines

Late (Parrots psuedoparalysis is an early sign)

Early or late congenital syph: tabes dorsalis

Late

Early or late congenital syph: Cluttons joints

Late (symmetric swelling of the knees

Are the Langerhans cells in LCH CD1a+ or not?

Yes they are positive for CD1a+

This type of LCH presents before age 2 as multiple small pink papules in a seborrheic distribution. Represents an acute disseminated form. Natal teeth

Letterer-Siwe-Disease | "Little sweetie disease"

Neonatal varicella occurs when mom is infected in what time frame of the pregnancy?

7 days before delivery to 2 days after delivery | Vs fetal varicella occurs when mom is infected in the first trimester

What is the clinical presentation of neonatal varicella?

Infant born with large blisters (Essentially like chicken pox just much more widespread) *difference between neonatal/congenital varicella and childhood varicella is that all the lesions in neonatal varicella are in the same stage of development vs childhood, they are all at different stages of development

What is the clinical presentation of fetal varicella?

Born with large stellate scars. Also limb paresis, chorioretinitis, hypoplasia

Neonatal acne caused by which organism?

Malassezia

Child with Down's develops a severe pustular reaction on the face, what lab should you order and what should you be concerned about?

CBC with diff - worried about leukemia! Can present as a pustular reaction on the face in Trisomy 21 patients

Patient presentation: - fever - exanthum - acral peeling - strawberry tongue - cervical lymphadenopathy - conjunctivitis What do you worry abt?

This is Kawasaki - need 5/6 to meet criteria So look our for cardiac aneurysm!! (In 1/4 of untreated pts)

Treatment for PLEVA

Oral Erythromycin

Genoderm with KP atrophicans (ulerythema ophyrogenes)

Noonan syndrome

Atrophoderma vermiculata a/w which genoderms

Rombo syndrome, Downs

Atrophoderma vermiculata BCC Trichoeps Acral vasodilation

Rombo

At what week of life does each appear: - skin - melanocytes - mature epidermis

skin: 6 weeks melanocytes: 8 weeks mature epidermis: 24 weeks

Peds Derm Flashcards

(91 cards)