Insights into clinical and diagnostic findings as well as treatment responses in patients with mucous membrane pemphigoid: A retrospective cohort study

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Samenvatting

Background: The variable clinical severity of mucous membrane pemphigoid (MMP) often leads to diagnostic and therapeutic delays.

Objective: To describe the characteristics of a large cohort of patients with MMP.

Methods: A retrospective review of clinical and diagnostic characteristics as well as treatment responses in 145 patients with MMP.

Results: Monosite involvement was seen in 41.4% and multisite involvement in 58.6% of the patients. The oral mucosa was affected in 86.9% of the patients, followed by the ocular mucosa (30.3%), skin (26.2%), genital mucosa (25.5%), nasal mucosa (23.4%), and pharyngeal and/or laryngeal mucosa (17.2%). Ocular disease developed during the disease course in 41.7% of patients with initially other mucosal site involvement. The malignancy rate was significantly higher in patients with autoantibodies against laminin-332 than in patients with MMP without laminin-332 autoantibodies (35.3% vs 10.9%, respectively; P = .007). Systemic immunosuppressive or immunomodulatory therapy was administered to 77.1% of the patients, mainly to patients with multisite (P

Limitations: Retrospective design.

Conclusion: Patients with MMP present with a heterogeneous clinical presentation, and new symptoms may develop during the disease course. Cancer screening should be considered for patients with MMP and, in particular, for those with autoantibodies against laminin-332.

Originele taal-2English
Pagina's (van-tot)48-55
Aantal pagina's8
TijdschriftJournal of the American Academy of Dermatology
Volume87
Nummer van het tijdschrift1
Vroegere onlinedatum9-dec-2021
DOI's
StatusPublished - jul-2022

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