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Loïc Vaillant, MD; Philippe Bernard, MD, PhD; Pascal Joly, MD, PhD; Catherine Prost, MD, PhD; Bruno Labeille, MD; Christophe Bedane, MD, PhD; Brigitte Arbeille, MD; Elisabeth Thomine, MD; Philippe Bertrand, MD; Catherine Lok, MD; Jean-Claude Roujeau, MD; for the French Bullous Study Group

Arch Dermatol. 1998;134:1075-1080.

Objective  To check the potential usefulness of clinical criteria for the diagnosis of bullous pemphigoid when state-of-the-art techniques such as Western immunoblotting, immunoprecipitation, and indirect immunofluorescence on salt-split skin or direct immunoelectron microscopy are not available.

Design  Comparison of the clinical criteria between 2 groups (with and without bullous pemphigoid) as defined by immunoelectron microscopy used as standard criterion, in a prospective study. Multivariate logistic regression analysis was carried out by including all items that were statistically significant (at P<.05 level) in univariate analysis.

Setting  Five dermatology departments in teaching hospitals.

Patients  The 231 patients studied had subepidermal autoimmune bullous diseases with linear IgG or C3 deposits in the basement membrane zone (157 with bullous pemphigoid, 33 with cicatricial pemphigoid, 30 with epidermolysis bullosa acquisita, 5 with lupus erythematosus, and 6 others). A second set of patients was used to calculate predictive values.

Results  The multivariate logistic stepwise analysis resulted in a final set of predictors that included only 4 items: absence of atrophic scars, absence of head and neck involvement, absence of mucosal involvement, and age greater than 70 years. No additional variables met the .05 significance level to enter into the model. If 3 of these 4 characteristics were present, a diagnosis of bullous pemphigoid could be made with a sensitivity of 90% and a specifity of 83%; these predictive values were calculated on a sample of 70 new cases.

Conclusions  With an estimated incidence of bullous pemphigoid among subepidermal autoimmune bullous diseases of 80%, the presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid, with a positive predictive value of 95%. Our set of' clinical criteria thus allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

From the Department of Dermatology, Hôpital Trousseau, Tours, France (Dr Vaillant); Department of Dermatology, Hôpital Dupuytren, Limoges, France (Drs Bernard and Bedane); Departments of Dermatology (Dr Joly) and Pathology (Dr Thomine), Hôpital C. Nicolle, Rouen, France; Department of Dermatology, Hôpital Saint Louis, Paris, France (Dr Prost); Department of Dermatology, Hôpital H. Mondor, Créteil, France (Dr Roujeau); Department of Dermatology, Hôpital Sud, Amiens, France (Drs Labeille and Lok); and Departments of Electron Microscopy (Dr Arbeille) and Biostatistics (Dr Bertrand), Hôpital Bretonneau, Tours.

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