J Am Acad Dermatol. 2021 Dec 9:S0190-9622(21)02958-3. doi: 10.1016/j.jaad.2021.11.061. Online ahead of print.
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 < .001), ocular (P < .001), and pharyngeal and laryngeal involvement (P = .002). The remaining patients (22.9%) received topical therapy. Adverse events were frequently reported.
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.