Patients with mucous membrane pemphigoid need multidisciplinary monitoring

Patients with mucous membrane pemphigoid should be monitored by a multidisciplinary team due to a risk for malignancy, according to a study.

“Mucous membrane pemphigoid (MMP) comprises a group of heterogeneous, autoimmune blistering diseases, with predominant mucosal involvement, characterized by autoantibodies directed against structural proteins — including bullous pemphigoid (BP) 180, BP230, laminin-332, type VII collagen and alpha6 and B4 integrin subunits — in the epidermal basement membrane zone,” Hanan Rashid, MD, of the department of dermatology at the Center of Blistering Diseases and the department of pathology at the University of Groningen, both in Groningen, the Netherlands, and colleagues wrote.

Researchers conducted a retrospective review of patients diagnosed with MMP between 2002 and 2019 at the Center for Blistering Diseases.

Treatment responses to dapsone, cyclophosphamide, azathioprine, mycophenolic acid and rituximab monotherapies or in combination with short-term prednisone were assessed for disease control and remission.

Additionally, predictors of malignancy were analyzed using binary logistic regression.

Of 145 patients with MMP, 60 (41.4%) had one mucosal site involvement, whereas 85 (58.6%) had multisite involvement.

A median diagnosis delay of 12 months (interquartile range, 21.8 months) was recorded, with no significant difference reported between monosite and multisite involvement due to diagnostic delay.

The most common area of involvement was oral mucosa with 86.9% of patients, followed by ocular mucosa (30.3%), skin (26.2%), genital mucosa (25.5%), nasal mucosa (23.4%) and pharyngeal and/or laryngeal mucosa (17.2%).

In patients with other mucosal site involvement, ocular disease developed in 41.7% and a significantly higher malignancy rate was recorded in those with vs. without autoantibodies against laminin-332 (35.3% vs. 10.9%).

A greater proportion of those with multisite vs. monosite involvement received systemic therapies (89% vs. 60.3%).

Those with ocular involvement and those with pharyngeal involvement with or without laryngeal involvement received systemic therapy more often than those without these mucosal site involvements.

“Symptoms in other mucosal sites in patients with MMP, such as ocular disease, might develop during the disease course,” the authors wrote. “Therefore, patients with MMP should be regularly monitored by a multidisciplinary team.”

Due to the risk for malignancy, especially in those with laminin-332 antibodies, cancer screening should also be considered, they added.