Clinical Features and Pathogenesis of Chronic Bullous Disease of Childhood

Abstract

Chronic bullous disease of childhood (CBDC) also known as linear IgA disease (LAD), although rare, is the most common acquired immunobullous disease of childhood. It shares the same immunohistochemical features as LAD in adults characterized by the deposition of IgA along the basement membrane zone which causes a subepidermal blister. The distinct clinical presentation of CBDC distinguishes it from other entities and will be reviewed in this chapter. The etiology of CBDC is most often idiopathic. Drug-induced linear IgA especially from vancomycin is more common in adults than children and may respond to stopping the medication culprit. Standard of care is treatment with dapsone, but other treatments have been used with varying success. Although mucous membrane involvement and scarring may occur in a subset of patients, the majority of children with this condition have a good prognosis, with remission expected within months to a few years.