Mucous membrane pemphigoid through the years in our Department

Abstract

Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic in ammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation.

There are autoantibodies against di erent components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6β4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.

Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.

Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.

Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the

Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the a ected mucous, evolution time until the diagnosis, direct immuno uorescence findings, clinical follow-ups and treatments. 

Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most a ected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response.

Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, di ering with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.