Vesiculopustular and erosive disorders in newborns and infants Flashcards
Epidemiology of erythema toxicum neonatorum?
Half of full term infants, >2500g; M=F
Ddx for eosinophilic spongiosis?
HAAPPIED H: Herpes Gestationis A: Arthropod Allergic contact P: Pemphigus, Pemphigoid, I: Incontinentia pigmenti, ETN Drug reaction
Timing for Erythema toxicum neonatorum?
24-48 hours after birth usually but can be seen from birth to 2 weeks.
Clinical presentation of Erythema toxicum neonatorum?
Erythematous macules, papules, pustules, and wheals.
DOES NOT OCCUR ON PALMS AND SOLES
Histology of Erythema toxicum neonatorum?
Subcorneal and intrafollicular eosinophilic pustules (wright’s stain of pusutles = eos)
The clinical course of Erythema toxicum neonatorum?
Self limited, resolves over several weeks
What systemic lab findings can be seen in Erythema toxicum neonatorum?
Peripheral blood sample can show eosinophilia
Epidemiology of Transient Neonatal Pustular Melanosis?
Term infants, MC in blacks (~5% of darkly pigmented newborns)
Timing of Transient Neonatal Pustular Melanosis?
Presents at birth or shortly after, but collarettes or hyperpigmentation can be a few days-weeks of age.
3 stages of Transient Neonatal Pustular Melanosis?
- Pustules w/o underlying erythema
- Collarettes of scale
- Hyperpigmentated macules
Most common distribution of Transient Neonatal Pustular Melanosis?
- face
- neck
- trunk
- extremities (palms, sole)
Histology of Transient Neonatal Pustular Melanosis?
- Subcorneal pustules w/ neuts, fibrin, and rarely eosinophils
- Wright’s stain of pustule fluid: neutrophils
Prognosis of Transient Neonatal Pustular Melanosis?
Self-limited and resolves over sever weeks
Subcorneal pustules DDX
CAT PISS
Candida
Acropustulosis of infancy
Transient Neonatal Pustular Melanosis
Pustular psoriasis
Impetigo
Sneddon-Wilkinson (and IgA pemphigus)
Staph Scalded skin
Epidemiology of miliaria crystallina?
15% of newborns
Most common locations of Miliaria Crystallina?
Forehead, upper trunk, and arms
Pathogenesis of Miliaria Crystallina?
Intracorneal obstruction of eccrine duct
Timing of Miliaria Crystallina?
Neonates and infants (hx of fever and overheating usually)
Clinical presentation of Miliaria Crystallina?
Fragile, clear-colored vesicles without underlying erythema
Self limited
Timing of Miliaria Rubra?
After first week of life
Pathogenesis of Miliaria Rubra?
Deeper intraepidermal obstruction of eccrine duct w/ inflammation
Causes of Miliaria Rubra?
Hx of overwarming, fever, or use of occlusive dressing or garment
Clinical presentation of Miliaria Rubra?
Erythematous papules w/ superimposed pustules typically concentrated in one or two areas
Most common location of Miliaria Rubra?
Intertriginous/occluded sites (neck, groin, axilla) most commonly
Histology of Miliaria Rubra?
Intraepidermal spongiosis and vesicles + chronic inflammatory infiltrate in dermis
What is the epidemiology of Neonatal Cephalic Pustulosis?
Appears within the first few weeks, resolves by 3 months. 20% of healthy newborns. M>F
Pathogenesis of Neonatal Cephalic Pustulosis?
- KOH may demonstrate Malassezia (subtype of neonatal acne, termed neonatal cephalic pustulosis)
- Other cases likely related to stimulation of sebaceous glands by maternal androgens or transient androgen production
Clinical of Neonatal Cephalic Pustulosis?
Inflammatory papules and pustules more common than comedones on the cheeks and nasal bridge common, but may occur anywhere on face/head/neck
Treatment for Neonatal Cephalic Pustulosis?
- Usually regresses over few months
- If mild, cleanse with gentle soap and water
- For more severe cases can consider ketoconazole cream or hydrocortisone 1-2.5% cream
If not regressing or if severe rule out neonatal acne
What do you need to think about in a baby w/ trisomy 21 and neonatal cephalic pustulosis?
Could also be a leukemoid reaction that can manifest as a severe pustular eruption on the face, mimicking neonatal acne.
Epidemiology of Infantile Acne?
Appears around 3-6mo, resolves by 2-3 years
M>F
Pathogenesis of infantile acne?
Due to hormonal imbalance (hyperandrogenism)
This is not from the mother as is often hypothesized, from the infants own androgen production
Clinical presentation of infantile acne?
- More severe and persistent compared with neonatal acne
- Comedones (primarily) and inflammatory lesions including occasionally deep cysts
- Can result in scarring
- Usually limited to the face
Treatment for infantile acne?
- Therapy is often necessary due to risk of scarring
- Same options as neonatal acne
- If occurs between ages 1-7yo (mid-childhood acne) and signs of pubertal development are noted (pubarche, thelarche, etc) an endocrinology evaluation should be considered along with the following laboratory analysis: DHEAS, androstenedione, 17-OH progesterone, and bone age
- May predict propensity towards future acne
Timing of Cutaneous Candidiasis?
Presents after first week of life
Clinical presentation of cutaneous candidiasis?
- Pink-red patches w/ satellite papules and pustules that favor diaper area or other intertriginous sites and face
- Can often have oral thrush
What is congenital candidiasis?
Less common than cutaneous candidiasis, candidiasis infxn in congenital infxn is acquired in utero
- Widespread vesiculopustular eruption, often involving palms and soles
- Nail dystrophy w/ yellowing and transverse ridging may be the only manifestation
- Characteristic yellow-white papules may be found on the umbilical cord
What is invasive fungal dermatitis?
Occurs in very low birth weight premature newborns “Invasive fungal dermatitis”
Can present as “burn like” erythema w/ desquamation and erosions
What are sucking blisters?
Occurs in 1 in every 250 newborns. Caused by vigorous sucking of the affected area in utero leading to Intact flaccid bullae, erosion, or superficial ulceration on non-inflamed base
- Isolated finding w/o associated issues
- Resolves over days to a week
Distribution of sucking blisters?
Radial forearm, wrist, hands, fingers
Epidemiology/onset of acropustulosis of infancy?
Onset is typically between birth and 2yo
It is most common in black infants boys
The etiology is unknown; some cases a/w preceding scabies infection
What is the clinical presentation of acropustulosis of infancy?
Recurrent crops of vesiculopustules over the palms, soles, and distal extremities
Smear findings for acropustulosis of infancy?
Neuts
Treatment for acropustulosis of infancy?
Systemic antihistamines and high potency topical corticosteroids
Typically resolves spontaneously by 3yo
Epidemiology and timing of Eosinophilic Pustular Folliculitis of Infancy?
Mean age of onset=6mo, M>F, presents at birth or days to weeks of age
Clinical presentation of Eosinophilic Pustular Folliculitis of Infancy?
Waxing and waning course over months with recurrent crops of pustules that remit eventually may be pruritic.
The pustules and erythema mainly involves the scalp and face but occasionally the trunk and extremities
Histology of Eosinophilic Pustular Folliculitis of Infancy?
Dense perifollicular mixed infiltrate w/ eosinophils
Labs for Eosinophilic Pustular Folliculitis of Infancy?
Can show eosinophilia
Prognosis of Eosinophilic Pustular Folliculitis of Infancy?
Resolves by 3 y/o
Clinical of Congenital and Neonatal Langerhans Cell Histiocytosis?
Starts at birth to a few days after
Skin limited form, rapidly self-healing; widespread (>solitary) red or purple-brown papulonodules +/- erosions, crust, and resolves weeks later
Can develop anywhere including palms and soles
Tests that need to be done in Congenital and Neonatal Langerhans Cell Histiocytosis?
R/o systemic involvement: PE, chem panel, LFT, CBC, Urine osmolality, abdominal US, Skeletal survey, CXR
What is the histology of congenital and neonatal Langerhans cell histiocytosis?
There is a proliferation of Langerhans cells in the superficial dermis with large ovoid cells w/ coffee bean nuclei
stains + for s100, CD1a, langerin
Genetics of Incontinentia pigmenti?
XLD loss of function mutation in nuclear factor-kappaB(NF-kB) essential modulator (NEMO;IKBKG)
Pathogenesis of IP?
- Mutation in NEMO prevents activation of NF-kB, a regulator of cell proliferation, inflammation, and TNF-alpha induced apoptosis
- Mutation in lethal in males; seen primarily in women and XXY (Klinefelter’s) males
- Functional mosaicism from lyonization in affected females (random inactivation of affected X chromosome) results in blaschkoid pattern of cutaneous involvement.
Clinical of IP?
IP is a neuroectoermal disorder that affects the skin, teeth (hypodontia/anodontia), CNS, and eyes
Cutaneous lesions are typically arranged in streaks and whorls, following blaschkoid pattern
What are the 4 morphologic states of IP?
Vesicular, verrucous, hyperpigmented, and hypopigmented.
you can also see patchy scarring alopecia at the vertex, areas of wooly hair, nail dystrophy, and anhidrosis with atrophic linear streaks.
What is the histology of the vesicular stage of IP?
Eosinophilic spongiosis; intraepidermal vesicles containing eosinophils; apoptotic keratinocytes in the epidermis
Keys points of the histology for each stage of IP?
- Vesicular Stage: eosinophilic spongiosis; intraepidermal vesicles containing eosinophils; apoptotic keratinocytes in the epidermis
- Verrucous stage: papillomatosis, hyperkeratosis, and acanthosis of the epi; apoptotic cells in epi forming squamous eddies
- Hyperpigmented stage: marked pigment incontinence w/ numerous melanophages in the dermis; apoptotic cells may be seen in the epidermis
- Hypopigmented stage: epidermal atrophy, loss of melanin in the basal layer, the complete absence of pilosebaceous units and eccrine glands; apoptotic cells may be seen in the epidermis
Can male babies be born with IP?
Generally not (x-linked and fatal to boy babies). However, women with missense mutations in NEMO (Milder phenotype of IP) can bear children (usually male) with hypohidrotic ectodermal dysplasia with immunodeficiency
Other clinical findings in IP?
Severely affected patients may develop seizures, developmental delay, and intellectual impairment, and decreased visual acuity/blindness (due to retinal vascular changes and optic atrophy)
Pathogenesis of Autosomal Dominant Hyperimmunoglobulin E (Hyper IgE) Syndrome
Primary immunodeficiency syndrome: AD, STAT3 mutations
–Signal transducer and activator of transcription 3 gene
Clinical of Autosomal Dominant Hyperimmunoglobulin E (Hyper IgE) Syndrome
Mean onset: 7 days old, Neonatal Papulopustular + vesicular eruption w/ crusting of face, scalp, neck, axillae, diaper area
- Elevated serum IgE levels (>2000), peripheral eosinophilia, “cold” abscesses, eczematous dermatitis, recurrent infections
- AR variant: DOCK8 deficiency
What is the most common organism to cause invasive fungal dermatitis in a neonate?
Candida albicans
