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We encountered a neonatal case of linear IgA bullous dermatosis. Only one other case of the disease diagnosed in the neonatal period has been reported, so we felt that it was important to describe this case.
Small vesicles first appeared on the face, hands, and legs of a Chinese full term baby boy on day 3 of life, which evolved into bullae on day 13. New bullae continued to erupt until day 18. By day 25, all the skin lesions had crusted, and skin healing was complete without scar formation. Besides skin eruption, the most overwhelming feature of the course was mucosal involvement. The infant presented with stridor on day 10 and went into respiratory failure requiring intubation. On day 30, bronchoscopy revealed a swollen larynx and a vesicle on the left ari-epiglottic fold. He was extubated on day 38 in the middle of a three week course of prednisolone. After extubation, stridor gradually subsided in a couple of weeks.
The diagnosis of linear IgA bullous dermatosis was made by skin biopsy on a bulla. Histological sections showed splitting of the skin at the dermo-epidermal junction with predominant polymorph infiltrate. Immunofluorescence showed a linear deposit of IgA at the dermo-epidermal junction. Staining for IgG and C3 was also positive.
Linear IgA bullous disease commonly occurs in childhood with onset from 6 months to 10 years.1 It classically runs a relapsing course with complete remission attained after puberty. The overall incidence of involvement of mucous membranes of the oral cavity, eyes, and external genitalia is 57%, 40%, and 72% respectively.1 However, the mucosal involvement is not life threatening.
The other neonatal case of linear IgA bulbous disease reported in the literature also showed serious mucosal involvement. It manifested as respiratory failure requiring treatment by extracorporeal membrane oxygenation, oesophageal dysmotility with choking during feeding, and blindness as a result of conjunctival scarring.2 In both these neonatal cases, complete remission was attained after the unsettled neonatal period. Hence, linear IgA bullous disease with onset in the neonatal period contrasts sharply with the classical presentation of the childhood disease in having serious mucosal involvement and a non-relapsing course.
We hope that our report serves as a reference for neonatologists and dermatologists who may encounter similar cases in the future.
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