vesiculopustular diseases of newborn Flashcards

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1
Q

Erythema toxicum neonatorum pathology?

Does it have a predilection for full term or pre term babies

when does it present after birth?

tx?

A

Erythematous macules, papules, pustules > vesicles, wheals, spares palms/ soles

vesiculopustular eruption characterized by eosinophilic infiltrate, expect intrafollicular, subcorneal or intraepidermal pustules with eosinophils, eosinophils will also be in the upper dermis as well

affects babies at 24-48 hrs

predilection for full term babies

self limited

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2
Q

Transient neonatal pustular melanosis

affects which type of baby?

When does it present?

What is seen on pathology?

A

Pustules without erythema, collarettes of scale, hyperpigmented macules

affects any region, MAY affect palm soles

affects 5% of dark pigmented babies

Presents at birth or shortly after (however collarettes/ hyperpigmentation may be few days-weeks)

Sterile subcorneal neutrophilic infiltrate, fibrin, pustules are straight up neutrophils

SELF RESOLVES

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3
Q

Miliaria AKA ?

A

Heat rash

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4
Q

What condition is seen here and how often does it affect newborns?

A

Miliaria crystallina - fragile vesicles, NO erythema

due to obst. of the eccrine sweat ducts as it courses through the corneum (clear, small, flaccid vesicles “dew drops”)

Miliaria is a common condition that affects up to 15% of newborns, and it is seen more frequently in warm climates.

Sweat collects beneath the stratum corneum, causing clear, small, flaccid vesicles that are often likened to “dew drops”

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5
Q

What is seen here and describe path briefly

What is seen on histology?

A

Miliaria Rubra, blockage of sweat ducts deeper into the spinous layer (leads to vesicles, _erythematous_ papules, pustules)

Histo findings: Eccrine duct obstruction leads to intraepidermal spongiosis, vesicles with chronic inflammatory infiltrate in dermis, dermal inflammation around occluded eccrine ducts

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6
Q

Name condition

Epidemiology (how many infants does it affect)

Pathogenesis?

A

Neonatal cephalic pustulosis - papules and pustules on an erythematous base

Affects chin, forehead, cheeks, eyelids, neck chest and scalp

affects 20-50% of term infants, onset within 2-3weeks of life

Path: inflammatory rxn to Malassezia

Tx: self limited versus topical imidazole/ hydrocort

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7
Q

What condition?

Male versus female?

Onset?

Pathogenesis?

Tx?

A

Infantile Acne

Male > female (RARE)

onset= 6wk-1yr

Androgen production in excess

Tx: topical retinoids/ BP, ABX

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8
Q

Two varieties of neonatal candidiasis

describe epidemiology of each

Pathogenesis and treatment difference

A

Neonatal : acquired during first week of life/ shortly after (during delivery usually)

Dx KOH

Tx: topical imidazole cream

Congenital → Uncommon, acquired in utero, though may appear as late as day 6 after birth

Path: risk factors → foreign body in uterus/ cervix, premature delivery, maternal hx of vaginal candidiasis

DX: KOH

Tx: Systemic antifungals

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9
Q

What is seen here?

etiology ?

self limiting?

tx?

path?

A

Acropustulosis of infancy → happens at 3-6 months of age, occasionally earlier (vesicles and pustules)

unknown etiology → scabies?

Pruritic acral vesiculopustular eruption

recurs q3-4 weeks, decreasing in frequency and eventually running its course

tx with systemic roids/ histamines

  • Path: subcorneal pustules w/ neuts +/- lymphocytes + eos*
  • No bacteria/ fungus*
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10
Q

what to think of in baby hand?

A

sucking blister

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11
Q

What is this?

A

Arthropod bites

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12
Q

What is this?

A

scabies

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13
Q

???

A

Epidermolysis bullosa simplesx

Birth to first few days or older

Mechanically induced blisters and erosions; depending on type: mucosal erosions, aplasia cutis congenita of anterior legs, scarring, milia, nail dystrophy

Widespread or limited, depending on type; most often extremities, especially hands/feet

Biopsy of induced blister for immunofluorescence antigen mapping ± electron microscopy; genetic analysis

Difficulty feeding, failure to thrive; occasionally corneal, respiratory tract or gastrointestinal (e.g. pyloric atresia) involvement; anemia

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14
Q

???condition>

A

acrodermatitis enteropathica

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15
Q

condition>

epidemiology?

Path?

Tx?

A

between birth and 15 months, usually seen around 6 months

possibly related to acropustulosis of infancy

Dense eosinophils around follicles w/ outer root sheath/ dermal interstitium

+/- peripheral eosinophilia

tx → mid potency steroids, antihistamines, tacrolimus, po dapsone/ abx in severe cases

Waxes/ wanes around 3 yerasrs

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16
Q

the clue to this diagnosis is in the color …

A

Neonatal lupus

17
Q

which diagnosis?

epidermiology

path?

Tx?

A

Congenital and neonatal langerhan cell histiocytosis

Path → clonal neoplastic disorder

60% BRAF, Map2k1, ERK activation

  • Langerhan cell (APC which migrates to and from epidermis)*
  • → densely proliferates in papillary dermis*

+S100, CD1a, CD207 (langerin stain)

tx→ only skin limited? resolves on its own

Vemurafinib for BRAF V600E

Refer to ONC

18
Q

describe the 4 variants to Langerhan cell histiocytosis

A

Letterer - Siwe → <2 yo, acute disseminated skin and visceral lesions, seborrheic papules/ pust/ vesicles in scalp, flexural areas, erosions and fissures, poor prognosis, osteolytic bone lesions, thrombocytopenia/ anemia

Hand Schuller-christian → 2-6 yo, TRIAD: osteolytic bone lesions, DI, exophthalmos

Congenital self-healing reticulohistiocytosis “Hashimoto-Pritzker” → birth to a few days, skin limited form, rapid resolution

Eosinophilic Granuloma → 7-12 YO, localized LCH = usually solitary ASYMPTOMATIC bone lesions, treat bone lesions with curettage?

19
Q

Which condition>?

Path?

Clinical findings?

A

Incontinentia Pigmenti

presents w/in 1-2 weeks of age

PATH: Defective NF-KB activation

Xlinked Dominant multisystem disease → often fatal

Vesicular, verrucous, hyperpigmented, atrophic/ hypopigmented

alopecia, nail and teeth dystrophy PEG TEETH*, CNS seizures, psychomotor retardation, occular → blindness

20
Q

Hyper IgE Syndrome

Clinical findings

Pathology/ what is the mutation

what do you seen on histo

A

Its a primary immunodeficiency, AD STAT3 mutation

clinically → papulopustular/ vesicular eruption + crusting on head shoulders/ diaper area ( its early extreme eczema)

Recurrent infections, coarse facial features, retention of primary teeth, osteopenia

Intraepidermal vesicles containing eosinophils, eosinophilic folliculitis

21
Q

Neonatal behcets

How do kids get it

Self limited?

Clinical findings?

A

infants born to behcets moms with active disease, immunoglobulins last about 3 months , self limited

ulcers on oral/ genitals, vesiculopustules, purpuric/necrotic skin, pathergy

22
Q

Which vesicular disease is associated with down syndrome

A

Vesiculopustular eruption in transient myeloproliferative disorder of Down syndrome

→ congenital lekuemoid rxn affecting 10% or so of babies with down syndrome

intraepidermal spongiotic vesiculopustules full of immature myeloid cells → vesicles on face, trunk, extremities

increases risk of AML in these kids, however it is self limited generally initially

23
Q

Congenital erosive and vesicular dermatosis affects which babies

Widespread erosions, vesicles, crusting, scalded skin like erythema, <75% BSA

Path?

A

Premature neonates

Epiderma necrosis, subepidermal versiculation, eroded epidermis with neuts/ dermal infiltrate

Tx: supportive care

24
Q

Restrictive Dermopathy

Pathogenesis

Clinical picture

Tx?

A

LMNA mutation

lethal neonatal laminopathy, also only happens in prematures

rigid tense skin, akinesia, hypokinesia, joint contractions

most newborns die of restrictive pulmonary disease

25
Q

eosinophilic pustular folliculitis of infancy

Similar to API? (*acropustulosis of infancy)

A

Similar but happens at around 6 months of age, includes scalp and occasional acral involvement

intraepidermal follicular based eosinophilic infiltrate

Tx Mid potency steroids, topical tacro, oral antihistamines

26
Q

Langerhan cell histiocysotis

Langerhan cells look like what and stain with what on histology?

Their presence in the epidermis is called ___

A

coffee bean nuclei, stain with S100/ CD1a/ Langerin

27
Q

Incontinentia pigmenti 4 phases of skin lesions

A
  1. Vesicular stage - yellow or clear vesicles in streaks that follow lines of blaschko, lasts 1-2 weeks, recurs for up to 1 year, facial sparing
  2. verrucous phase #2, hyperkeratotic linear plaques
  3. linear and whorled gray brown hyperpigmentation, on the trunk
  4. subtle, atrophic, hypopigmented thin streaks favoring the calves

Additional cutaneous findings include wooly hair, nail dystrophy, anhidrosis, hypodontia or conical teeth, seizures, delayed psychomotor development, spastic paralysis, occular disease (retina)

28
Q

Autosomal dominant hyper IgE syndrome

What mutation?

What physical descriptions?

After the neonatal period, what are characteristic findings?

A

Stat3

Rash = neonatal papulopustular and vesicular eruption with crusting on face, scalp, neck and axilla/ diaper

cold abscesses, pruritic chronic eczematous dermatitis, recurrent pneumonia, osteopenia, retention of deciduous teeth