Bullous pemphigoid: Three main clusters defining 3 outcome profiles

• Published in: Journal of the American Academy of Dermatology Vol. 87; no. 2; pp. 359 - 365
• Main Authors: Guerrois, Florine, Hassan, Elsa, Bettuzzi, Thomas, Seta, Vannina, Goulvestre, Claire, Jelti, Lamia, Belmondo, Thibaut, Wolkenstein, Pierre, Aractingi, Selim, Ingen-Housz-Oro, Saskia, Dupin, Nicolas
• Format: Journal Article
• Language: English
• Published: United States 01.08.2022
• Subjects: Autoantibodies; Autoantigens; Blister; Dystonin; Humans; Non-Fibrillar Collagens; Pemphigoid, Bullous; Retrospective Studies; phenotype; bullous pemphigoid; anti-BP180; anti-BP230; relapse; disease control; blistering disease
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/j.jaad.2022.04.029

Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. Its presentation is polymorphic. To investigate different clinical and biological profiles of BP. 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. 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. Retrospective study without immunoelectron microscopy. We identified 3 different BP clusters, including one corresponding to severe BP180 BP230 BP with features common to mucous membrane pemphigoid.