Chronic bullous dermatosis of childhood: relapse after puberty

Chronic bullous dermatosis of childhood (CBDC) is a distinctive subepidermal blistering disorder that characteristically involves the perioral area, lower trunk, inner thighs and genitalia. Direct immunofluorescence shows a linear band of IgA at the dermoepidermal junction (DEJ). The eruption usually begins before the age of 6 years and is typically described as self-limiting, clearing with a few months or years. Chronic bullous dermatosis of childhood has many clinical and immunopathological similarities with adult linear IgA disease (LAD) and it has been suggested that they represent different manifestations of the same disease. We report a case of CBDC which started at 2 years of age and which has relapsed twice, first at age 6 years and then again, after puberty, at age 17 years.