Dermatology Flashcards

1
Q

Aplasia cutis associated with?

benign form is AD

lesions re-epithelialize

A

limb defects
cleft lip/palate
epidermolysis bullosa
chromosomal anomalies (T13, 4p)

If lesion is large/unusual appearing, consider head imaging

could have neurologic complications

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Lab findings in infant with E.tox

A

presence of numerous eosinophils by Wright-staining of the pustule
Peripheral eosinophilia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

3 stages of neonatal pustular melanosis

A
  1. small, non-inflammatory pustules without erythematous base, usually present at birth
  2. ruptured pustules with scale surrounding hyperpigmented macule
  3. hyperpigmented macules (can last up to 3 months)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What causes miliaria?

A

obstruction of sweat glands leading to sweat retention

think heat rash

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What causes milia?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What do the vesicles in incontinentia pigementi contain?

A

Eosinophils
clear or yellow color

stages:
1. vesicles on an erythematous base
2. verrucous hyperkaratotic lesions
3. HYPERchromic spots
4. HYPOchromic atrophic lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What other abnormalities are associated with incontigentia pigmenti?

X-linked dominant, majority female (lethal for males in utero), rare

A

seizures
mental deficiency
spastic paralysis
abnormal dentition
alopecia
nail hypoplasia
retinal vascular proliferation
80% central nervous system, eye, dentition

no treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is Kasabach-Merritt syndrome?

A

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 well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How do you treat a hemangioma?

A
  1. internal or system effects: oral propranolol followed by topical beta-blocker to prevent rebound
  2. consider systemic corticosteroids if unsuccesful

topical beta-blocker: if small, superficial, not complex

extreme cases: may required pulse-dye laser therapy or surgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

preterm female, benign vascular cell tumor

A

hemangioma

face is most common location

F>M = term
F=M = preterm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

vascular tumor

A

proliferating endothelium
benign- infantile hemangioma or congenital hemangioma

locally aggressive: Kaposiform hemangioendothelioma

malignant: angiosarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

vascular malformation

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

infantile hemangioma

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the sign called where the epidermis can be detached by gentle traction?
Disease association?

A

Nikolsky sign

Staph scalded skin

due to EXOTOXIN for staph aureus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Disease process for staph scalded skin

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Newborn in first few days of life presenting with blisters that rupture easily leaving honey-colored crusts

A

Bullous impetigo

due to staph

genital region, abdomen, inner thigh

bullae- polymorphonuclear cells
Tx: antibiotics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Presentation for junctional epidermolysis bullosa

A
  1. atrophic scarring
  2. blisters at DERMAL and EPIDERMAL jxn- lamina lucida (basement membrane)
  3. Dystrophic nails
  4. oral AND anal mucosal involvement
  5. AR
  6. most with short life span
  7. electrolytes abnormal
  8. PYLORIC STENOSIS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Presentation for simplex (epidermolytic) EB

A
  1. majority without scaring
  2. intra-epidermal blisters
  3. feet, hands, scalp
  4. AD
  5. hemorrhagic bullae heal quickly
  6. mild course
19
Q

Presentation for dystrophic (dermolytic) EB

A
  1. scarring
  2. intra-dermal blisters
  3. secondary to decrease in type VII collagen
  4. increased collagenase
  5. AR (more severe) and AD
  6. cycle of blistering, infection, scarring
  7. hair loss, anemia, dysphagia, poor growth
20
Q

Two types of infantile hemangiomas

A
  1. RICH: rapidly involuting, completely formed at birth, involutes by age 14
  2. NICH: non-involuting, completely formed at birth, no changes throughout life
21
Q

Posterior fossa brain malformations (75%)
facial Hemangiomas (>90%)
cerebroArterial abnormality (~40%)
Cardic anomalies (~25%, MC aorta)
Eye abnormalities (~10%)
Sternal clefting (~7%)

A

Neurocutaneous disorder, developmental defect between 8-10 weeks gestation
F>M

hemangiomas: “beard” distribution

22
Q

What is a nevus flammeus simplex?

AKA salmon patch

A

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

23
Q

Newborn lying on the side, half the body turns bright red in contrast to pale other half, demarcation line present

A

Harlequin color change

Etiology: secondary to temperature imbalance with abnormal autonomic regulation of cutaneous vessels

24
Q

Infant with pink pupils, yellow/white hair, decreased skin pigment, photophobia, nystagmus

A

Albinism
Secondary to deficiency of tyrosinase (tyrosine–> dopamine pathway blocked –> limited melanin production

25
Q

Another name for partial albinism

A

piebaldism

AD
normal pigmented areas adjacent to amelanocytic regions
absent or deformed melanocytes

26
Q

What do you call linear, whorl-like, streaky hypopigmented macules

A

Blaschko’s lines

Dx: hypomelanosis of ito= incontinentia pigmenti achromians

palms, soles, scalp not usually involved

27
Q

Describe the typical appearance of a collodion infant

A
  1. preterm
  2. brownish-yellow translucent shiny skin (cellophane-like membrane)
  3. 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

28
Q

Infant with “blood hound appearance”

A

Cutis laxa= generalized elastolysis
normal wound healing

29
Q

Infant born to a mother with autoantibodies anti-Sjogren’s A (anti-Ro), anti-Sjogrens B (anti-La), anti-U1RNP antibodies

A

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

30
Q

Conditions associatd with pustules

A

Staph
Listeria
GBS

31
Q

Conditions associated with vesicles

A

early stages of Staph aureus
Listeria
GBS
Pseudomonas
herpes
varicella-zoster

32
Q

Conditions associated with bullae

A

Staph
can also be Syphilis (palms and soles)

33
Q

Conditions associated with maculopapules

A

staph and strep
fungal
measles
enterovirus
rubella
syphilis (palms/soles, desquamating)

34
Q

Conditions associated with cellulitis

A

Strep

35
Q

Impetigo

A

Strep
less likely: staph, E.coli

36
Q

Conditions associated wtih abscesses

A

Staph
strep
E.coli
Candida

37
Q

Conditions associated with pyoderma gangrenosum

A

Pseudomonas

yellow-green pustules

38
Q

Conditions associated with blueberry muffin rash

results from dermal hematopoiesis

A

rubella
CMV
Coxsackie B2
Parvovirus B19
twin-twin transfusion
hemolytic disease of the neonate
hereditary spherocytosis
neoplastic infiltrative diseases
congenital leukemia

39
Q

Pathognomonic skin leasions in congenital syphilis

A

hemorrhagic bullae and petechiae on palms and soles which spread to trunk and extremities

40
Q

infant with multiple grey-white smooth 1-2mm nodules on the maxillary-alveolar ridge, lesions are not found 1 month later

A

Bohn nodules

Ebstein pearls = hard palate

41
Q

Describe an epulis

A

single, soft, pedunculated nodule over the gingiva of the anterior maixllary ridge
multiple may occur
excision required

42
Q

translucent lesion that arises from the floor of the mouth and may appear blue
may rupture spontaneously

A

ranula

43
Q

Diseases associated with collodion infant

A

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

44
Q

Another name for Sturge Weber Syndrome (SWS)?

A

Encephalotrigeminal angiomatosis