A study of benign chronic bullous dermatosis of childhood and comparison with dermatitis herpetiformis and bullous pemphigoid occurring in childhood

Eighteen patients with benign chronic bullous dermatosis of childhood were studied and the findings compared with those of dermatitis herpetiformis (twenty-two cases) and bullous pemphigoid (five cases) beginning in childhood. The patients with benign chronic bullous dermatosis of childhood had a moderately pruritic bullous eruption with maximal involvement of the pelvic and perioral regions which tended to occur at an earlier age than either dermatitis herpetiformis or bullous pemphigoid. In contrast to dermatitis herpetiformis one-third of the cases with benign chronic bullous dermaiosis of childhood went into remission. Evidence of coeliac disease was only found in the dermatitis herpetiformis group. Surprisingly both diseases shared HLA-B8. A linear BMZ band of IgA was detected on direct immunofluorescence in all but one of the cases with benign chronic bullous dermatosis of childhood and circulating antibodies were detectable in two-thirds. Routine histopathology was of little value in distinguishing between benign chronic bullous dermaiosis of childhood and dermatitis herpetiformis or bullous pemphigoid. Several paradoxes have yet to be explained before it can be determined whether benign chronic bullous dermatosis of childhood is a variant of dermatitis herpetiformis or linear IgA disease.