Mucous Membrane Pemphigoid: Diagnosis and Sequel

Introduction: Mucous membrane pemphigoid is a disease characterized by subepidermal bullae primarily involving the oral and ocular mucous membranes. In its pathogenesis there is linear deposition of IgG, IgA, and C3 along the epithelial basement membrane zone. Its prevalence is estimated between 1/12,000 and 1/20,000 in general population and its clinical manifestations are varied, being invasive and aggressive.

Objectives: This study aims to expose the difficulties encountered to get a concrete diagnosis, which enables to reduce the risk of limiting sequel.

Resumed Report: A 34-year-old man presents with rhinorrhea, hoarseness, hemoptysis, dyspnea, synechia between the septum and turbinates, and supraglottic stenosis. Underwent tracheostomy and investigation with biopsy, which enabled diagnoses of leishmaniasis, Wegener granulomatosis, and vasculitis of Behçet but which did not respond to treatment. Finally, after new biopsy, the diagnosis of mucous membrane pemphigoid was obtained, confirmed by immunofluorescence. A treatment was initiated at intervals until disease control with the use of hyperimmune immunoglobulin. This resulted in long period of research, which brought irreversible consequences to the patient.

Conclusion: Being a rare disease and with multiple forms of presentation, the diagnosis may be delayed, resulting sequels. Therefore, the knowledge about this disease and the multidisciplinary approach to diagnosis and treatment are essential for a good result.

Keywords: larynx, mucous membrane pemphigoid, supraglottic stenosis, cicatricial pemphigoid.