Dermatology Flashcards
Aplasia cutis associated with?
benign form is AD
lesions re-epithelialize
limb defects
cleft lip/palate
epidermolysis bullosa
chromosomal anomalies (T13, 4p)
If lesion is large/unusual appearing, consider head imaging
could have neurologic complications
Lab findings in infant with E.tox
presence of numerous eosinophils by Wright-staining of the pustule
Peripheral eosinophilia
3 stages of neonatal pustular melanosis
- small, non-inflammatory pustules without erythematous base, usually present at birth
- ruptured pustules with scale surrounding hyperpigmented macule
- hyperpigmented macules (can last up to 3 months)
What causes miliaria?
obstruction of sweat glands leading to sweat retention
think heat rash
What causes milia?
small epidermal inclusion cysts - retension of keratin and sebaceous material in the folicles
resolve by 1-3 months of age
if in mouth = epstein’s pearls
What do the vesicles in incontinentia pigementi contain?
Eosinophils
clear or yellow color
stages:
1. vesicles on an erythematous base
2. verrucous hyperkaratotic lesions
3. HYPERchromic spots
4. HYPOchromic atrophic lesions
What other abnormalities are associated with incontigentia pigmenti?
X-linked dominant, majority female (lethal for males in utero), rare
seizures
mental deficiency
spastic paralysis
abnormal dentition
alopecia
nail hypoplasia
retinal vascular proliferation
80% central nervous system, eye, dentition
no treatment
What is Kasabach-Merritt syndrome?
multiple leasions that resemble hemangiomas or single large rapidly growing hemangioma type lesion (hemangioendotheliomas)
can lead to:
high-output heart failure
disseminated intravascular coagulation
thrombocytopenia
How do you treat a hemangioma?
- internal or system effects: oral propranolol followed by topical beta-blocker to prevent rebound
- consider systemic corticosteroids if unsuccesful
topical beta-blocker: if small, superficial, not complex
extreme cases: may required pulse-dye laser therapy or surgery
preterm female, benign vascular cell tumor
hemangioma
face is most common location
F>M = term
F=M = preterm
vascular tumor
proliferating endothelium
benign- infantile hemangioma or congenital hemangioma
locally aggressive: Kaposiform hemangioendothelioma
malignant: angiosarcoma
vascular malformation
normal endothelial turnover
caused by errors in vasculogenesis
slow-flow: capillary malformation, venous malformation, lymphatic malformation
fast-flow: arterial malformation, arteriovenous fistula, AV malformation
grow proportionally with infant
infantile hemangioma
benign vascular endothelial cell tumor
presence of GLUT-1 transporter differentiates this from other vascular tumors
increase until 6 mo then stable and start to decrease in size by ~ 1 yo
What is the sign called where the epidermis can be detached by gentle traction?
Disease association?
Nikolsky sign
Staph scalded skin
due to EXOTOXIN for staph aureus
Disease process for staph scalded skin
initially bright red in the face
severe bullous eruptions
bullae are flaccid and rupture easily
shed in large sheets, crusting around mouth and eyes
conjunctivitis is common
no scaring, rapid recovery
fluid from bullae is STERILE
tx: antibiotics, fluid management
Newborn in first few days of life presenting with blisters that rupture easily leaving honey-colored crusts
Bullous impetigo
due to staph
genital region, abdomen, inner thigh
bullae- polymorphonuclear cells
Tx: antibiotics
Presentation for junctional epidermolysis bullosa
- atrophic scarring
- blisters at DERMAL and EPIDERMAL jxn- lamina lucida (basement membrane)
- Dystrophic nails
- oral AND anal mucosal involvement
- AR
- most with short life span
- electrolytes abnormal
- PYLORIC STENOSIS
Presentation for simplex (epidermolytic) EB
- majority without scaring
- intra-epidermal blisters
- feet, hands, scalp
- AD
- hemorrhagic bullae heal quickly
- mild course
Presentation for dystrophic (dermolytic) EB
- scarring
- intra-dermal blisters
- secondary to decrease in type VII collagen
- increased collagenase
- AR (more severe) and AD
- cycle of blistering, infection, scarring
- hair loss, anemia, dysphagia, poor growth
Two types of infantile hemangiomas
- RICH: rapidly involuting, completely formed at birth, involutes by age 14
- NICH: non-involuting, completely formed at birth, no changes throughout life
Posterior fossa brain malformations (75%)
facial Hemangiomas (>90%)
cerebroArterial abnormality (~40%)
Cardic anomalies (~25%, MC aorta)
Eye abnormalities (~10%)
Sternal clefting (~7%)
Neurocutaneous disorder, developmental defect between 8-10 weeks gestation
F>M
hemangiomas: “beard” distribution
What is a nevus flammeus simplex?
AKA salmon patch
Vascular macule resulting from capillary malformation
Neck, forehad, upper eyelids
Irregular borders, blanching
Usually fade within first year of age
Exaggerated in patients with BWS
Newborn lying on the side, half the body turns bright red in contrast to pale other half, demarcation line present
Harlequin color change
Etiology: secondary to temperature imbalance with abnormal autonomic regulation of cutaneous vessels
Infant with pink pupils, yellow/white hair, decreased skin pigment, photophobia, nystagmus
Albinism
Secondary to deficiency of tyrosinase (tyrosine–> dopamine pathway blocked –> limited melanin production
Another name for partial albinism
piebaldism
AD
normal pigmented areas adjacent to amelanocytic regions
absent or deformed melanocytes
What do you call linear, whorl-like, streaky hypopigmented macules
Blaschko’s lines
Dx: hypomelanosis of ito= incontinentia pigmenti achromians
palms, soles, scalp not usually involved
Describe the typical appearance of a collodion infant
- preterm
- brownish-yellow translucent shiny skin (cellophane-like membrane)
- desquamation (parchment-like) complete by 2-3 weeks of age, skin beneath is red
Caused by defective cutaneous barrior function, increased insensible water loss, increased risk of hypothermia, infection (increased risk of pneumonia from aspiration of squamous cells)
Ectropion, eclabion, abnormal digits
Infant with “blood hound appearance”
Cutis laxa= generalized elastolysis
normal wound healing
Infant born to a mother with autoantibodies anti-Sjogren’s A (anti-Ro), anti-Sjogrens B (anti-La), anti-U1RNP antibodies
Neonatal lupus syndrome
Round or elliptical erythematous lesions with central clearing, associated with scale
Rash worsens with exposure to phototherapy and sunlight
Present in first 3 months until 6-8 months when maternal antibodies no longer present
Conditions associatd with pustules
Staph
Listeria
GBS
Conditions associated with vesicles
early stages of Staph aureus
Listeria
GBS
Pseudomonas
herpes
varicella-zoster
Conditions associated with bullae
Staph
can also be Syphilis (palms and soles)
Conditions associated with maculopapules
staph and strep
fungal
measles
enterovirus
rubella
syphilis (palms/soles, desquamating)
Conditions associated with cellulitis
Strep
Impetigo
Strep
less likely: staph, E.coli
Conditions associated wtih abscesses
Staph
strep
E.coli
Candida
Conditions associated with pyoderma gangrenosum
Pseudomonas
yellow-green pustules
Conditions associated with blueberry muffin rash
results from dermal hematopoiesis
rubella
CMV
Coxsackie B2
Parvovirus B19
twin-twin transfusion
hemolytic disease of the neonate
hereditary spherocytosis
neoplastic infiltrative diseases
congenital leukemia
Pathognomonic skin leasions in congenital syphilis
hemorrhagic bullae and petechiae on palms and soles which spread to trunk and extremities
infant with multiple grey-white smooth 1-2mm nodules on the maxillary-alveolar ridge, lesions are not found 1 month later
Bohn nodules
Ebstein pearls = hard palate
Describe an epulis
single, soft, pedunculated nodule over the gingiva of the anterior maixllary ridge
multiple may occur
excision required
translucent lesion that arises from the floor of the mouth and may appear blue
may rupture spontaneously
ranula
Diseases associated with collodion infant
Lamellar ichthyosis- due to mutation in transglutaminase-1 gene (14q11)
Nonbullous congenital ichthyosiform erythroderma
Recessive x-linked ichthyosis
Neutral lipid storage disease (Dorfman-Chanarin syndrome)
Trichothiodystrophy
Gaucher’s
Sjogren-Larsson syndrome
at risk for HYPERNATREMIC dehydration
Another name for Sturge Weber Syndrome (SWS)?
Encephalotrigeminal angiomatosis
