Arch Dermatol
-
Multicenter Study
Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.
The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 x 10(6). ⋯ Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for short-term clinical studies in France.