A comparative study of benign chronic bullous dermatosis of childhood and linear IgA dermatosis of adults

Abstract

Benign chronic bullous dermatosis of childhood (BCBDC) is an apparently self‐limiting vesiculo‐bullous disease showing linear IgA deposits in the basement membrane zone and which seems to occur exclusively in children. Its relationship to linear IgA dermatosis of adults (LAD) is uncertain and for this reason we studied 28 patients with BCBDC (onset before puberty) and compared them with 18 patients with LAD (onset after puberty). No significant clinical, HLA, histological or immunopathological differences were found between the two disorders. Surprisingly, nine patients in each group had conjunctival scarring which in some produced a cicatricial pemphigoid‐like appearance and in one case of BCBDC resulted in blindness. Remissions with loss of IgA from the skin occurred in patients from both groups. However, the disease duration varied considerably (BCBDC 2–28 and LAD 1–40 years) with six BCBDC patients having had their disease for over 10 years and five of them passing puberty. All patients (six BCBDC, eight LAD) studied by immuno‐electronmicroscopy showed localization of IgA beneath the basal lamina. Direct and indirect immunofluorescence studies using suction blisters also gave similar results in both groups. As a result of this study many of our former views on BCBDC have been revised and we now believe that both BCBDC and LAD are manifestations of the same disease.