Vesiculobullous Diseases Non-Infectious Etiology. Vesiculobullous Diseases. Infectious Etiology

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Dylan Lloyd
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1 Vesicles and Pustules in the Newborn Julian Trevino, M.D. Associate Professor Boonshoft School of Medicine Department of Dermatology Vesicles and Pustules in the Newborn Represent wide spectrum of disorders Several conditions presenting with these findings are truly life- threatening Many conditions with these findings are innocuous and self-limitedlimited All newborns and infants with vesicles and pustules should be evaluated with: Thorough history and physical exam Appropriate laboratory evaluation(s) Vesiculobullous Diseases Non-Infectious Etiology Transient neonatal pustular melanosis crystallina and rubra (neonatal acne ) Eosinophilic pustular folliculits Incontinentia pigmenti Neonatal herpes gestationis Neonatal pemphigus Epidermolysis bullosa Congenital self-healing reticulohistiocytosis Vesiculobullous Diseases Infectious Etiology Scabies Neonatal candidiasis Staphylococcal scalded skin syndrome Herpes simplex Congenital varicella Congenital syphilis 20-60% of term infants Less frequently in preterm neonates ~5% Types of lesions: erythematous macules, wheals, papules and pustules Sites of predilection - face, trunk, proximal arms and buttocks Palms and soles almost never affected (due to lack of pilosebaceous units)

are truly life- threatening Many conditions with these findings are innocuous and self-limitedlimited All newborns and infants with vesicles and pustules should be evaluated with: Thorough history

2 Unknown Has been postulated to be hypersensitivity reaction based on eosinophilic response Most begin during second 24 hours of life Rarely present at birth May begin any time from birth to 2 weeks Exacerbations and remissions occur during first few weeks of life Usually based on clinical appearance of the rash in an otherwise healthy term infant Atypical cases: Scraping of pustules stained with Wright s stain demonstrate large number of eosinophils Peripheral eosinophilia in 15% of cases Self-limitinglimiting Requires no treatment Reassure parents as to benign/noninfectious nature of condition Transient Neonatal Pustular Melanosis () 2 2 to 5% of term black newborns 0.6% of term Caucasian newborns Equal numbers of boys and girls affected

of pustules stained with Wright s stain demonstrate large number of eosinophils Peripheral eosinophilia in 15% of cases Self-limitinglimiting Requires no treatment Reassure parents as to

3 Pustules or pigmented macules, with/without a surrounding collarette of scale present singly or in combination Located on forehead, chin, neck, back, hands and feet, including palms and soles Unknown Lesions present at birth and progress to a brownish crust or rupture leaving a fine white collarette of scale Found in clusters or individually New lesions usually do not appear after birth Resolves in 24 to 48 hours Usually made clinically based on lesion morphology, time of onset, and absence of other findings Gram stain - demonstrates neutrophils, rare eosinophils and absence of bacteria Wright and Giemsa stain - demonstrates neutrophils and rare eosinophils Skin findings are not correlated with maternal infection or neonatal infection Prognosis is excellent with self-resolution; however, hyperpigmented macules may last for several weeks to months before resolving Treatment is non-essential

in 24 to 48 hours Usually made clinically based on lesion morphology, time of onset, and absence of other findings Gram stain - demonstrates neutrophils, rare eosinophils and absence of bacteria

4 A common dermatosis of the neonate and infant In warm climates, may be present in up to 15% of newborns Two types of miliaria occur in the newborn period -- milaria rubra ( prickly heat ) and crystallina; miliaria rubra is the most frequently seen Equal among sexes and races Vesicles, pustules or papules in crops Occurs on the face, trunk and interiginous areas Usually presents in term and pre-term neonates greater than ten days old Infrequently miliaria crystallina presents at birth; miliaria rubra more common after first week of life Follows excessive warming in incubator, fever, occlusive dressings, or inappropriately i warm clothing Obstruction of the eccrine duct, followed by leakage of the eccrine sweat into the skin Extracellular polysaccharide produced by some strains of Staph epidermidis may obstruct sweat delivery crystallina is occasionally present at birth i rubra more common after first week of life Usually based on lesion location, time of onset, and history of excessive warming In cases where diagnosis is uncertain a skin biopsy can be performed

and pre-term neonates greater than ten days old Infrequently miliaria crystallina presents at birth; miliaria rubra more common after first week of life Follows excessive warming in incubator, fever,

5 Acropustulosis of Infancy Resolves spontaneously, but may have recurrences with rubra The lesions resolve in 1 to 3 days with shedding of keratinous plugs Treatment involves cool baths and removal of excess clothing Seen in less than 1% of newborns Increased di in African-American American males Association with atopy in some patients and families Intensely pruritic vesicles and/or pustules on palms and soles, dorsa of hands and feet, sides of fingers and toes, ankles, wrists, and occasionally the chest, back, and abdomen More common form occurs following scabies infestation Usually follows scabies that has been severe or prolonged in duration Unknown Lesions may be present at birth, but more often develop in the first weeks or months of life The lesions appear in crops every 2 to 4 weeks; individual lesions last 5-10 days; course may last 1-2 years

palms and soles, dorsa of hands and feet, sides of fingers and toes, ankles, wrists, and occasionally the chest, back, and abdomen More common form occurs following scabies infestation Usually

6 Must differentiate from scabies Giemsa, Wright and Gram stains Reveal numerous neutrophils, and occasional eosinophils Bacteria, scabies mites/eggs/feces are absent KOH - negative for hyphae Usually remits spontaneously within 1 to 2 years Symptomatic treatment includes ld systemic antihistamines and potent topical steroids Severe disease may be treated with Dapsone 1-2 mg/kg/day (requires laboratory evaluation for G-6- PD deficiency and frequent monitoring) (neonatal acne ) May be seen in up to 20% of newborns Papulopsutular facial eruption usually concentrated on cheeks; also may involve forehead, chin, eyelids, neck, upper chest and scalp Comedones are absent Likely related to stimulation of sebaceous glands by maternal androgens or transient adrenal and gonadal androgen production Several authors have proposed Malassezia furfur and M. sympoidalis as causes of this condition

laboratory evaluation for G-6- PD deficiency and frequent monitoring) (neonatal acne ) May be seen in up to 20% of newborns Papulopsutular facial eruption usually concentrated on cheeks; also may

7 May be present at birth Mean age of onset is 2-3 weeks Condition remits spontaneously after several weeks Usually made on clinical presentation Giemsa-stained stained smears demonstrate fungal spores as well as neutrophils TREATMENT Topical imidazole creams (e.g., ketoconazole) Low-potency topical steroids Condition remits spontaneously after several weeks Over 700 cases reported 97% females due to X-linked dominant inheritance - vesicular stage Four Stages Vesicular - erythematous macules, papules, vesicles and bullae in a linear arrangement following the lines of Blaschko on extremities, trunk and scalp Verrucous - streaks of hyperkeratotic papules and pustules Hyperpigmentation - hyperpigmented macules and patches along Blaschko s lines Hypopigmentation - hypopigmentation of previously hyperpigmented areas,with or without follicular atrophy

, ketoconazole) Low-potency topical steroids Condition remits spontaneously after several weeks Over 700 cases reported 97% females due to X-linked dominant inheritance - vesicular stage Four Stages

8 - hyperpigmented stage - hypopigmented stage ADDITIONAL FINDINGS Hair - scarring alopecia in 30% Nails - dystrophic changes in 5 to 10 % Teeth - anodontia, peg/conical teeth in 66% Eyes - strabismus, cataracts, optic atrophy, retinal vascular changes and retrolental mass in 25 to 35 % CNS - seizures, mental retardation and spastic paralysis in 30% X-linked dominant genodermatosis localized to gene locus Xp11.21 or possibly the Xq28 region NEMO gene (NFKB essential modulator) defect Skin lesions present at birth in 50%; occur within 2 weeks in 90% of cases Vesicular - birth to 2 weeks Verrucous - 2 to 6 weeks Hyperpigmentation - 3 to 6 months Hypopigmentation - 2nd to 3rd decades

paralysis in 30% X-linked dominant genodermatosis localized to gene locus Xp11.

9 Clinical and histologic correlation Detailed family history and complete skin exam of mother and female siblings Normal life span Complete physical exam Ophthalmology exam upon diagnosis Dental exam by 1 year Neurology exam upon diagnosis Genetic counseling for family

Complete physical exam Ophthalmology exam upon diagnosis Dental

Newborn Skin Rashes. References

Newborn Skin Rashes. References Newborn Skin Rashes COMPETENCIES: The resident should be able to: Recognize presenting signs and symptoms of a neonatal herpes simplex virus infection Recognize erythema toxicum and characteristics of