Bullous pemphigoid: Three main clusters defining 3 outcome profiles

Abstract

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. Its presentation is polymorphic.

Objective: To investigate different clinical and biological profiles of BP.

Methods: We conducted a retrospective 2-center study including all BP patients seen between January 1, 2015, and February 28, 2021. We performed hierarchical clustering on principal components.

Results: Three clusters were identified. Patients in cluster 1 (n = 155) were older than those in clusters 2 (n = 89) and 3 (n = 35; P < .0001), more frequently presented pauci-bullous BP (n = 63 [41%] vs 14 [16%] and 2 [6%], respectively; P < .0001) and had anti-BP230 antibodies in 87% of cases. More than 100 blisters were observed in 14 patients (40%) from cluster 3, versus 3 (2%) from cluster 1 and 0 (0%) from cluster 2 (P < .0001). Frequency of mucosal involvement was higher in cluster 3 (n = 32 [91%, including epiglottis in 40%] vs 11 [7%] and 34 [38%]; P < .0001). In clusters 2 and 3, 70% and 74% of patients had antibodies targeting only BP180. Those in cluster 3 received more lines of systemic treatment and experienced more relapses.

Limitations: Retrospective study without immunoelectron microscopy.

Conclusion: We identified 3 different BP clusters, including one corresponding to severe BP180⁺ BP230^- BP with features common to mucous membrane pemphigoid.

Keywords: anti-BP180; anti-BP230; blistering disease; bullous pemphigoid; disease control; phenotype; relapse.