3 clusters define outcome profiles in bullous pemphigoid

A recent study has identified three different clusters of bullous pemphigoid (BP), which includes one corresponding to severe BP180⁺ BP230⁻ with features common to mucous membrane pemphigoid.

A retrospective two-centre study was conducted to examine different clinical and biological profiles of BP. All BP patients seen between 1 January 2015 and 28 February 2021 were included. The researchers, led by Dr Florine Guerrois from the Cochin Hospital in Paris, France, carried out hierarchical clustering on principal components.

Guerrois and colleagues identified three clusters. Patients in cluster 1 (n=155) were older than those in clusters 2 (n=89) and 3 (n=35; p<0.0001) and more frequently presented pauci-bullous BP (41 percent vs 16 percent and 6 percent, respectively; p<0.001). Eighty-seven percent of patients in cluster 1 also had anti-BP230 antibodies.

More than 100 blisters developed in 14 patients (40 percent) from cluster 3 compared with only three (2 percent) from cluster 1 and none (0 percent) from cluster 2 (p<0.0001). In addition, cluster 3 (n=32) had a higher frequency of mucosal involvement (91 percent [including epiglottis in 40 percent] vs 7 percent and 38 percent; p<0.0001).

Moreover, 70 percent of patients in cluster 2 and 74 percent in cluster 3 had antibodies targeting only BP180. Those in cluster 3 received more lines of systemic treatment and had more relapses.

This study was limited by its retrospective design without immunoelectron microscopy.

“BP is the most common autoimmune blistering disorder,” the researchers said. “Its presentation is polymorphic.”