3 NEONATAL CASES OF EPIDERMOLYSIS-BULLOSA HERPETIFORMIS (DOWLING-MEARA TYPE) WITH SEVERE EROSIVE SKIN-LESIONS

We describe three neonates who had large eroded areas of skin on their extremities. The clinical course and ultrastructural findings were co nsistent with a diagnosis of epidermolysis bullosa herpetiformis (Dowl ing-Meara type). In each case blisters developed around eroded areas a fter birth and enlarged centrifugally in a herpetiform fashion. One pa tient died of sepsis at 8 days of age. In the two survivors blister fo rmation subsided gradually within 1 year. Ultrastructural studies conf irmed intraepidermal blister formation associated with spheric aggrega tes of tonofilaments in the lower epidermis. Spheric aggregates were a lso found in clinically uninvolved skin.