Bullous pemphigoid: Three main clusters defining 3 outcome profiles - 16/07/22

Doi : 10.1016/j.jaad.2022.04.029

Florine Guerrois, MD ^a, Elsa Hassan, MD ^b, Thomas Bettuzzi, MD ^b,^c, Vannina Seta, MD ^a,^d, Claire Goulvestre, MD ^e, Lamia Jelti, MD ^b,^f, Thibaut Belmondo, MD ^f,^g, Pierre Wolkenstein, MD, PhD ^b, Selim Aractingi, MD, PhD ^a,^h, Saskia Ingen-Housz-Oro, MD ^b,^c,^f,^⁎ , Nicolas Dupin, MD ^a,^f,^h
^a Dermatology Department, AP-HP, Cochin Hospital, Paris, France
^b Dermatology Department, AP-HP, Henri Mondor Hospital, Créteil, France
^c EpiDermE (Épidémiologie en Dermatologie et Évaluation des Thérapeutiques), Université Paris-Est Créteil Val de Marne (UPEC), Créteil, France
^d Cenpetence Center for Autoimmune Blistering Diseases MALIBUL, AP-HP, Cochin Hospital, Paris, France
^e Immunology and Biology Department, AP-HP, Cochin Hospital, Paris, France
^f Competence Center for Autoimmune Blistering Diseases, MALIBUL, AP-HP, Henri Mondor Hospital, Créteil, France
^g Immunology and Biology Department, AP-HP, Henri Mondor Hospital, Créteil, France
^h Laboratoire de Biologie Cutanée, Institut Cochin, Inserm U1016, Université de Paris, France

^∗Correspondence to: Saskia Ingen-Housz-Oro, MD, Service de Dermatologie, Hôpital Henri Mondor, AP-HP, Hôpitaux de Paris, France.Service de DermatologieHôpital Henri MondorAP-HPHôpitaux de ParisFrance

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.

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Key words : anti-BP180, anti-BP230, bullous pemphigoid, blistering disease, disease control, phenotype, relapse

Abbreviations used : BP, ELISA, IEM, MMP

Plan

Covariables of interest

	Drs Guerrois and Hassan are cofirst authors. Drs Aractingi, Ingen-Housz-Oro, and Dupin contributed equally to this article.
	Funding sources: None.
	IRB approval status: Reviewed and approved by the ethics committee of Henri Mondor Hospital on May 27th, 2021 (IRB 00011558 no. 2021-117).
	Reprints not available from the authors.

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Vol 87 - N° 2

P. 359-365 - août 2022 Retour au numéro

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Bullous pemphigoid: Three main clusters defining 3 outcome profiles - 16/07/22

Abstract

Background

Objective

Methods

Results

Limitations

Conclusion

Plan

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