Challenges and advances in ocular mucous membrane pemphigoid (OMMP); from pathogenesis to treatment strategies.

Abstract

Purpose: Mucous membrane pemphigoid (MMP) is a systemic autoimmune condition characterized by blistering and cicatrization, predominantly affecting mucous membranes, including those lining the esophagus, oropharynx, nasal cavity, trachea, conjunctiva, and genitalia. Ocular mucous membrane pemphigoid (OMMP) is observed in approximately 70% of MMP cases. This study aims to review the pathophysiology, clinical manifestations, diagnosis, treatment, and complications of OMMP.

Methods: A literature search was conducted using MEDLINE and EMBASE databases.

Results: OMMP is characterized by the deposition of autoantibodies along the basement membrane zone of mucous membranes, particularly affecting the conjunctival epithelium. OMMP manifests as chronic ocular discomfort, inflammation, conjunctival scarring, eyelid abnormalities, and visual impairment. Given the extensive range of similar conditions, including drug-induced pseudo-pemphigoid and paraneoplastic conjunctival cicatrization, challenges in differential diagnosis may arise. The clinical diagnosis of OMMP is supported by confirmatory biopsy with histopathology and immunofluorescence studies. The mainstay of management includes systemic immunomodulatory medications and anti-inflammatory agents, tailored to disease severity. Surgical interventions may be necessary, although caution is warranted due to the risk of exacerbating OMMP. Prompt diagnosis and treatment are essential to halt disease progression and prevent vision loss. Complications of OMMP include corneal disorders, lid disorders, and vision disturbances. A comprehensive understanding of OMMP aids in timely intervention and improved patient outcomes.

Conclusion: OMMP is a bilateral, chronic, progressive, relapsing-remitting condition. Early diagnosis and treatment of OMMP are necessary to prevent disease progression. The management of OMMP varies according to the severity of the disease, but often involves both medical control of the underlying inflammatory process and subsequent surgical correction of residual anatomical changes.