Three 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 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.
Furumura
M
M
Department of Dermatology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Imayama
S
S
Hori
Y
Y
D001768:Blister; D002675:Child, Preschool; D003896:Desmosomes / Q000648:ultrastructure; D004817:Epidermis / Q000473:pathology; D016110:Epidermolysis Bullosa Simplex / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007382:Intermediate Filaments / Q000648:ultrastructure; D015603:Keratinocytes / Q000473:pathology; D008297:Male; D012867:Skin / Q000473:pathology