vesiculopustular diseases of newborn Flashcards
Erythema toxicum neonatorum pathology?
Does it have a predilection for full term or pre term babies
when does it present after birth?
tx?
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
Transient neonatal pustular melanosis
affects which type of baby?
When does it present?
What is seen on pathology?
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
Miliaria AKA ?
Heat rash
What condition is seen here and how often does it affect newborns?
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”
What is seen here and describe path briefly
What is seen on histology?
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
Name condition
Epidemiology (how many infants does it affect)
Pathogenesis?
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
What condition?
Male versus female?
Onset?
Pathogenesis?
Tx?
Infantile Acne
Male > female (RARE)
onset= 6wk-1yr
Androgen production in excess
Tx: topical retinoids/ BP, ABX
Two varieties of neonatal candidiasis
describe epidemiology of each
Pathogenesis and treatment difference
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
What is seen here?
etiology ?
self limiting?
tx?
path?
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*
what to think of in baby hand?
sucking blister
What is this?
Arthropod bites
What is this?
scabies
???
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
???condition>
acrodermatitis enteropathica
condition>
epidemiology?
Path?
Tx?
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
the clue to this diagnosis is in the color …
Neonatal lupus
which diagnosis?
epidermiology
path?
Tx?
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
describe the 4 variants to Langerhan cell histiocytosis
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?
Which condition>?
Path?
Clinical findings?
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
Hyper IgE Syndrome
Clinical findings
Pathology/ what is the mutation
what do you seen on histo
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
Neonatal behcets
How do kids get it
Self limited?
Clinical findings?
infants born to behcets moms with active disease, immunoglobulins last about 3 months , self limited
ulcers on oral/ genitals, vesiculopustules, purpuric/necrotic skin, pathergy
Which vesicular disease is associated with down syndrome
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
Congenital erosive and vesicular dermatosis affects which babies
Widespread erosions, vesicles, crusting, scalded skin like erythema, <75% BSA
Path?
Premature neonates
Epiderma necrosis, subepidermal versiculation, eroded epidermis with neuts/ dermal infiltrate
Tx: supportive care
Restrictive Dermopathy
Pathogenesis
Clinical picture
Tx?
LMNA mutation
lethal neonatal laminopathy, also only happens in prematures
rigid tense skin, akinesia, hypokinesia, joint contractions
most newborns die of restrictive pulmonary disease
eosinophilic pustular folliculitis of infancy
Similar to API? (*acropustulosis of infancy)
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
Langerhan cell histiocysotis
Langerhan cells look like what and stain with what on histology?
Their presence in the epidermis is called ___
coffee bean nuclei, stain with S100/ CD1a/ Langerin
Incontinentia pigmenti 4 phases of skin lesions
- 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
- verrucous phase #2, hyperkeratotic linear plaques
- linear and whorled gray brown hyperpigmentation, on the trunk
- 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)
Autosomal dominant hyper IgE syndrome
What mutation?
What physical descriptions?
After the neonatal period, what are characteristic findings?
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
