Clinical Criteria for the Diagnosis of Bullous Pemphigoid: A Reevaluation according to Immunoblot Analysis of Patient Sera

Abstract

Background: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. Objective: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. Methods: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. Results: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5·10^–4). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. Conclusion: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.

Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

“…Alternatively, (5) if a patient's serum was not available for testing, the diagnosis was based on the presence of 3 of 4 well-established clinical criteria for BP. The latter allow the diagnosis of BP to be made with high sensitivity, specificity and positive predictive value [21]. It is noteworthy that management of the patients has been quite uniform and homogenous during the studied period.…”

Three Cases of Bullous Pemphigoid Associated with Dipeptidyl Peptidase-4 Inhibitors - One due to Linagliptin

“…The clinical diagnosis of BP [12,13] was confirmed via histological and DIF analysis of the perilesional skin of a second set of biopsies, which showed a linear deposit of immunoglobulin G and C3 along the basement membrane zone. Enzyme-linked immunosorbent assay tests for specific BP180 and BP230 antibodies were not conducted because they are currently not available at our center.…”

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

“…The diagnosis of typical BP is assessed by the association of suggestive clinical criteria that were shown to be sensitive and specific [1,13], combined to the results of histology and direct immunofluorescence. Following French national recommendations for the diagnosis of BP [2], immunological tests are recommended but often not necessary for the diagnosis of typical situations.…”