PATIENTS with multisite mucous membrane pemphigoid (MMP) frequently experienced persistent disease activity at oral and ocular sites, preventing simultaneous complete remission despite improvements across all affected areas, according to a retrospective cohort study. The findings highlight the challenges of achieving comprehensive disease control and suggest that treatment response should be assessed according to individual anatomical sites.
The study evaluated 77 patients with immunopathologically confirmed multisite MMP treated at a tertiary dermatology and ophthalmology referral centre between January 2013 and January 2025. All participants had disease affecting at least two anatomical sites and a minimum of 12 months of post-treatment follow-up.
The cohort included 55 females (71%), with a median age at diagnosis of 66 years. High-risk disease involving ocular, laryngeal, oesophageal, or anogenital sites was present in 68 patients (88%).
Oral involvement was most common, affecting 73 patients (95%), followed by ocular disease in 51 (66%) and nasal involvement in 38 (49%).
Only 35 patients (45%) achieved simultaneous multisite complete remission (mCR), defined as complete remission across all affected anatomical sites regardless of ongoing treatment. Median time to mCR was 32.0 months.
Despite universal clinical improvement, 64 patients (83%) achieved complete remission at one or more anatomical sites. However, persistent oral and ocular disease frequently prevented patients from achieving mCR.
Relapse following site-level complete remission was also common. Among the 64 patients who achieved remission at one or more sites, 28 (44%) subsequently experienced relapse. Oral mucosa was the most frequent site of relapse, affecting 18 patients (64%), followed by ocular mucosa in 14 (50%). Some patients experienced recurrence across multiple anatomical sites.
These findings suggest that multisite MMP may behave differently across affected anatomical locations, with persistent activity at particular mucosal surfaces limiting overall disease control.
Rituximab-based regimens and Janus kinase (JAK) inhibitor therapy were associated with high rates of clinical improvement. However, the study found that rituximab-based regimens were associated with lower mCR rates than previously published MMP cohorts.
Serious adverse events occurred most frequently with cyclophosphamide monotherapy and JAK inhibitor therapy, highlighting the importance of considering treatment-associated safety alongside clinical response.
The researchers concluded that fewer than half of patients with multisite MMP achieved simultaneous remission, despite favourable site-level responses. The findings support a site-specific approach to understanding disease activity and highlight the need for standardised multisite remission outcomes in future research.
Reference
McAleer L et al. Site-specific treatment and remission patterns in multisite mucous membrane pemphigoid. JAMA Dermatol. 2026;DOI:10.1001/jamadermatol.2026.4256.
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