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Site- and autoantigen-specific characteristics of mucous membrane pemphigoid

JAMA Dec 02, 2021

van Beek N, Kridin K, Bühler E, et al. - In patients with mucous membrane pemphigoid (MMP), screening for malignant neoplasm could be potentially beneficial as in these patients, malignant neoplasms were found to be linked with ocular disease and anti-laminin 332 reactivity.

  • This retrospective cohort study included 154 patients with MMP (mean [SD] age at diagnosis, 66.2 [13.8] years), of whom 125 (81.2%), 61 (39.6%), 34 (22.1%), and 16 (10.4%) had lesions involving the oral, ocular, nasal, and genital mucosae, respectively, and 35 (22.7%) showed cutaneous involvement.

  • Oral mucosa was the most frequently involved mucosal surface in MMP and the most frequently targeted antigen was BP180 (90 patients [58.4%]).

  • An inverse association of ocular disease with oral (adjusted odds ratio [aOR], 0.02) and nasal (aOR, 0.20) involvement was observed.

  • A 13-fold increased risk of malignant neoplasm (aOR, 13.07) was observed in relation to ocular disease.

  • An association of anti-laminin 332 reactivity with malignant neoplasm (aOR, 23.27) was identified, whereas anti-BP180 NC16A immunoglobulin G seropositivity was linked with absence of ocular lesions (aOR, 0.09).

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