Incidence and Mortality of Mucous Membrane Pemphigoid in France

Abstract Background Data on the incidence and mortality rates of mucous membrane pemphigoid (MMP) are limited and outdated. Objectives This study aimed to estimate the incidence and standardized mortality ratios (SMR) of MMP and identify prognostic factors for mortality. Methods We screened patients with MMP diagnosed in 20 dermatology departments in France between 2000 to 2021, using the French national database of rare diseases. The incidence rate was calculated based on the number of inhabitants in the corresponding administrative regions and patients’ vital status was obtained from the national institute of demography (INSEE) death register. Results A total of 663 patients (376 women, 287 men) with a mean age of 70.57 ± 13.58 years were included. Annualized incidence rates progressively increased over the years, from 1.38 cases/million inhabitants/year in 2012 to a peak of 3.86 in 2019. The highest annualized incidence rate was observed in patients aged between 80 and 85 years (9.91 cases/million inhabitants/year; 95% CI =6.43 to 15.28). The 1-, 3- and 5-year SMRs were 1.00 (95% CI = 0.73 to 1.33), 1.05 (95% CI = 0.85 to 1.26), and 1.01 (95% CI = 0.85 to 1.18), respectively. A higher SMR was observed in patients with ear, nose, and throat (ENT) involvement (SMR=1.26, 95% CI = 1.00 to 1.55). Predictors of mortality included age (Hazard ratio [HR]=3.65 for ages 75-85 years vs <65 years, 95% CI =1.67 to 7.99; p=0.001, and HR=11.94 for ages >85 years, 95% CI =5.40 to 26.41; p<0.0001), cancer (HR=2.66, 95% CI = 1.75 to 4.06; p<0.0001), and diabetes (HR=1.80, 95% CI = 1.17 to 2.77; p=0.008). Patients with ocular-only involvement (HR=1.96, 95% CI = 0.95 to 4.05; p=0.07) and ENT involvement (HR=1.63, 95% CI = 0.96 to 2.78; p=0.07) had a trend towards a higher risk of mortality compared with oral ± skin forms of MMP. Conclusions The incidence rate of MMP in France has more than doubled over the last decade. Ocular-only and ENT involvement were associated with a higher risk of mortality.

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Journal
British Journal of Dermatology
Published
2026-05-09
DOI
https://doi.org/10.1093/bjd/ljag180
Primary Topic
Autoimmune Bullous Skin Diseases
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article
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article

Incidence and Mortality of Mucous Membrane Pemphigoid in France

Louise Loridon, P. Modiano, M. Viguier, Emmanuelle Tancrède‐Bohin et al.
British Journal of Dermatology
Autoimmune Bullous Skin Diseases
article

Incidence and Mortality of Mucous Membrane Pemphigoid in France

Louise Loridon, P. Modiano, M. Viguier, Emmanuelle Tancrède‐Bohin, Julien Grosjean, C. Lepelletier, C. Becquart, F. Lombart, C. Morice, N. Litrowski, G. Jeudy, Marine Chastagner, S. Debarbieux, C. Abasq, Alexis Lefebvre, D Jullien, Billal Tedbirt, Christophe Bédane, Marie-Aleth Richard, Saskia Oro, Gaëlle Quéreux, Catherine Picard-Dahan, Pascal Joly, Céline Muller, André Gillibert, Vivien Hébert, Nicolas Dupin, Sophie Duvert-Lehembre, Anne Pham-Ledard
article en

Abstract

Abstract Background Data on the incidence and mortality rates of mucous membrane pemphigoid (MMP) are limited and outdated. Objectives This study aimed to estimate the incidence and standardized mortality ratios (SMR) of MMP and identify prognostic factors for mortality. Methods We screened patients with MMP diagnosed in 20 dermatology departments in France between 2000 to 2021, using the French national database of rare diseases. The incidence rate was calculated based on the number of inhabitants in the corresponding administrative regions and patients’ vital status was obtained from the national institute of demography (INSEE) death register. Results A total of 663 patients (376 women, 287 men) with a mean age of 70.57 ± 13.58 years were included. Annualized incidence rates progressively increased over the years, from 1.38 cases/million inhabitants/year in 2012 to a peak of 3.86 in 2019. The highest annualized incidence rate was observed in patients aged between 80 and 85 years (9.91 cases/million inhabitants/year; 95% CI =6.43 to 15.28). The 1-, 3- and 5-year SMRs were 1.00 (95% CI = 0.73 to 1.33), 1.05 (95% CI = 0.85 to 1.26), and 1.01 (95% CI = 0.85 to 1.18), respectively. A higher SMR was observed in patients with ear, nose, and throat (ENT) involvement (SMR=1.26, 95% CI = 1.00 to 1.55). Predictors of mortality included age (Hazard ratio [HR]=3.65 for ages 75-85 years vs <65 years, 95% CI =1.67 to 7.99; p=0.001, and HR=11.94 for ages >85 years, 95% CI =5.40 to 26.41; p<0.0001), cancer (HR=2.66, 95% CI = 1.75 to 4.06; p<0.0001), and diabetes (HR=1.80, 95% CI = 1.17 to 2.77; p=0.008). Patients with ocular-only involvement (HR=1.96, 95% CI = 0.95 to 4.05; p=0.07) and ENT involvement (HR=1.63, 95% CI = 0.96 to 2.78; p=0.07) had a trend towards a higher risk of mortality compared with oral ± skin forms of MMP. Conclusions The incidence rate of MMP in France has more than doubled over the last decade. Ocular-only and ENT involvement were associated with a higher risk of mortality.

British Journal of Dermatology
Ikerbasque (ES), IE University (ES), Inserm (FR), Université de Bourgogne (FR), Lyon College (US), Hotel Dieu Hospital (CA), Saint Louis University Hospital (US), Hôpital Charles-Nicolle (FR), Hôpital Cochin (FR), Normandie Université (FR), Hôpital Saint-André (FR), Institut Jacques Monod (FR), CHU Dijon Bourgogne (FR), Hôpital Edouard Herriot (FR), Hôpital Robert-Debré (FR), Hôpitaux Universitaires Henri-Mondor (FR), Centre Hospitalier Régional Universitaire de Brest (FR), Institut Cochin (FR), Maison des Sciences sociales et des Humanités de Dijon (FR), Hôpital Bichat-Claude-Bernard (FR), Centre Hospitalier Universitaire de Rouen (FR), Hôpital Claude Huriez (FR), Centre Hospitalier de Valenciennes (FR), Hôpital Dupuytren (FR), Hôpital de la Timone (FR), Hôpital Saint-Vincent-de-Paul (FR), Université de Rouen Normandie (FR), Université de Caen Normandie (FR)
Good health and well-being
Openalex Percentile: Top 5%
Autoimmune Bullous Skin Diseases
DOISourceOpenAlex
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