By Gary M. White, MD
Courtesy of Charlie Rosenberg, MD
Chronic bullous disease of childhood is an autoimmune blistering disease occurring in prepubertal children characterized by a linear deposition of IgA along the basement membrane zone.
Sometimes there is a prodromal sore throat and fever. Large bulla often in rosettes or in clusters of "jewels" in a prepubertal child is characteristic of CBDC. Annular, sausage or polycyclic shapes are common. The perioral and genital area are often affected. DIF shows linear IgA along the BMZ. Gluten sensitive enteropathy is not associated.
Mucosal lesions may occur in up to 64%. In long standing disease, ocular scarring can occur.
DIF of perilesional skin shows linear IgA along the DEJ. IIF often is positive for IgA when the disease is active.
Dapsone 2 mg/kg/day or sulphapyridine are used. Systemic steroids may be added if needed. The disease usually remits before puberty. Dicloxicillin 40 mg/kg TID worked dramatically well in 3 children. Sometimes, prednisone may be started initially while one checks the biopsy, IF, and G6PD. Then, dapsone is begun, e.g. 0.5 mg/kg and slowly titrated up till control is reached. Then, the prednisone can be tapered. Colchicine 0.5 mg BID has also been used in children [PD 1999;16;50].
Most cases remit within 2 years, and almost all by puberty, although cases going beyond puberty have been reported.
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