Evaluation of Clinical Criteria for Diagnosis of Bullous Pemphigoid

Abstract

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 PϽ.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 erythem-atosus, 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.

Spectrum of autoimmune blistering dermatoses in Tunisia: an 11‐year study and a review of the literature

“…Another possible explanation is that BP may be more prevalent in an occidental series because of the longer life expectancy; BP is considered a disease of elderly adults. However, this well‐known particularity found in the literature 24,25 is not confirmed in the present study as well as in previous Tunisian ones 13 as it seems that BP occurs preferentially in younger patients with a male predilection.…”

Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort

“…13,14,[26][27][28][29] Diagnosis of BP can be made with high specificity and sensitivity in patients with linear IgG and ⁄or C3 deposits along the dermoepidermal junction when three of four clinical criteria are present: age > 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. 30,31 In contrast to these latter reports, we have here noted that almost 20% and 15% of the patients have neck ⁄head involvement and mucosal lesions, respectively. Future studies are needed to confirm whether the presence of neck and head involvement really represents a criterion useful for dismissing the diagnosis of BP.…”

Clinical and Immunologic Factors Associated With Bullous Pemphigoid Relapse During the First Year of Treatment

“…Although our study has some limitations, diagnostic bias cannot be excluded but seems unlikely because all included patients fulfilled the clinical criteria used in previous studies. 25 In the present study, the absence of major selection bias is supported by the fact that the mean age, mean (SD) number of new blisters daily (18 [30]), and 1-year mortality are comparable to those of other recent French studies. 9,10, 22,50 As well, the rates of positive results of anti-BP180 and anti-BP230 ELISA were 90% and 60%, respectively, also quite similar to those found in previous studies.…”