Immunological features and factors associated with mucocutaneous bullous pemphigoid – a retrospective cohort study

Sascha Ständer, Enno Schmidt, Detlef Zillikens, Ralf J. Ludwig, Khalaf Kridin

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Background and objectives: The features of bullous pemphigoid (BP) patients presenting with mucosal lesions are not established. We aimed to elucidate the clinical and immunological features of BP patients with mucosal involvement, and to identify factors associated with mucosal lesions. Patients and methods: A retrospective study encompassing all consecutive patients diagnosed with BP throughout the years 2009–2019 in a tertiary referral center. Results: The study encompassed 273 patients with BP, of whom 31 (11.4 %) presented with mucosal lesions. The oral mucosa was the most frequently affected mucosal surface (71.0 %), followed by the genital (25.8 %) and the nasal (22.6 %) mucosae. Relative to other patients with BP, patients with mucosal involvement had a more prominent palmoplantar involvement (67.7 % vs. 37.2 %; P = 0.001); lower seropositivity rate (18.2 % vs. 54.2 %; P = 0.027) and lower levels (29.3 ± 64.5 vs. 129.5 ± 304.4 U/ml; P = 0.016) of anti-BP230 autoantibodies; and decreased peripheral eosinophil counts (760.0 ± 638.6 vs. 1296.3 ± 1013.7; P < 0.001). Absence of anti-BP230 autoantibodies (OR, 5.32; 95 % CI, 1.07–26.32; P = 0.026) and lack of peripheral eosinophilia (OR, 4.31; 95 % CI, 1.14–16.39; P = 0.021) were associated with the presence of mucosal involvement in BP. Conclusions: Mucosal involvement is present in a notable subgroup of patients with BP and is associated with the absence of both anti-BP230 antibodies and peripheral eosinophilia.

Original languageEnglish
Pages (from-to)1289-1295
Number of pages7
JournalJDDG - Journal of the German Society of Dermatology
Volume19
Issue number9
DOIs
StatePublished - Sep 2021

Bibliographical note

Publisher Copyright:
© 2021 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.

Funding

Clinical Research Unit Pemphigoid Diseases (KFO 303) and Cluster of Excellence Precision Medicine in Chronic Inflammation (EXC 2167), both funded by Deutsche Forschungsgemeinschaft.

FundersFunder number
Deutsche Forschungsgemeinschaft

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