Bullous pemphigoid: Three main clusters defining 3 outcome profiles

Area of Science:

• Dermatology
• Immunology
• Autoimmune Diseases

Background:

• Bullous pemphigoid (BP) is a common autoimmune blistering skin disease with diverse clinical presentations.
• Understanding these variations is crucial for effective patient management and treatment strategies.

Purpose of the Study:

• To investigate and define distinct clinical and biological profiles within bullous pemphigoid patient cohorts.
• To identify potential subgroups that may benefit from tailored therapeutic approaches.

Main Methods:

• A retrospective analysis of bullous pemphigoid patients from two centers between January 2015 and February 2021.
• Hierarchical clustering on principal components was employed to identify patient subgroups based on clinical and serological data.

Main Results:

• Three distinct bullous pemphigoid clusters were identified.
• Cluster 1 comprised older patients with pauci-bullous BP and anti-BP230 antibodies. Cluster 3 presented with severe blistering, high mucosal involvement (including epiglottis), and antibodies targeting BP180, showing features similar to mucous membrane pemphigoid.
• Clusters 2 and 3 predominantly featured antibodies targeting BP180, with cluster 3 patients experiencing more relapses and requiring more intensive treatment.

Conclusions:

• The study successfully delineated three distinct bullous pemphigoid patient clusters.
• A significant subgroup (cluster 3) exhibits severe disease, extensive mucosal involvement, and a specific autoantibody profile (BP180+, BP230-), suggesting overlap with mucous membrane pemphigoid.
• These findings highlight the heterogeneity of bullous pemphigoid and the need for personalized treatment strategies.