Three neonatal cases of epidermolysis bullosa herpetiformis (Dowling-Meara type) with severe erosive skin lesions.

Abstract

We describe three neonates who had large eroded areas of skin on their extremities. The clinical course and ultrastructural findings were consistent with a diagnosis of epidermolysis bullosa herpetiformis (Dowling-Meara type). In each case blisters developed around eroded areas after birth and enlarged centrifugally in a herpetiform fashion. One patient died of sepsis at 8 days of age. In the two survivors blister formation subsided gradually within 1 year. Ultrastructural studies confirmed intraepidermal blister formation associated with spheric aggregates of tonofilaments in the lower epidermis. Spheric aggregates were also found in clinically uninvolved skin.