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Philippe Bernard, MD; Loïc Vaillant; Bruno Labeille, MD; Christophe Bedane, MD; Brigitte Arbeille; Jean-Paul Denoeux, MD; Gérard Lorette, MD; Jean-Marie Bonnetblanc, MD; Catherine Prost, MD

Arch Dermatol. 1995;131(1):48-52.

Abstract
Background and Design
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 3.55x10⁶.

Results
Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases.

Conclusion
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 shortterm clinical studies in France.

(Arch Dermatol. 1995;131:48-52)

Author Affiliations
the Bullous Diseases French Study Group

From the Departments of Dermatology, Hôpital Dupuytren, Limoges, France (Drs Bernard, Bedane, and Bonnetblanc); Hôpital Trousseau, Tours, France (Drs Vaillant and Lorette); Hôpital Sud, Amiens, France (Drs Labeille and Denoeux); Hôpital Saint-Louis, Paris, France (Dr Prost); and the Electron Microscopy Unit, Hôpital Trousseau, Tours, France (Dr Arbeille).

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