Ocular mucous membrane pemphigoid: a review

• Published in: Immunologic research Vol. 67; no. 2-3; pp. 280 - 289
• Main Authors: Taurone, Samanta, Spoletini, Marialuisa, Ralli, Massimo, Gobbi, Pietro, Artico, Marco, Imre, Laszlò, Czakò, Cecília, Kovàcs, Illés, Greco, Antonio, Micera, Alessandra
• Format: Journal Article
• Language: English
• Published: New York Springer US 01-06-2019; Springer Nature B.V
• Subjects: Allergology; Autoimmune diseases; Biomarkers; Biomedical and Life Sciences; Biomedicine; Biopsy; Blistering; Blisters; Bullous pemphigoid; Conjunctiva; Conjunctiva - immunology; Conjunctiva - metabolism; Conjunctiva - pathology; Conjunctivitis - diagnosis; Conjunctivitis - etiology; Conjunctivitis - metabolism; Cornea; Cytokines; Disease; Disease Management; Disease Susceptibility; Disruption; Drug therapy; Epithelium; Extracellular matrix; Fibroblasts; Fibrosis; Growth factors; Humans; Immunology; Immunomodulation; Inflammation; Inflammatory response; Internal Medicine; Medicine/Public Health; Pemphigoid, Benign Mucous Membrane - diagnosis; Pemphigoid, Benign Mucous Membrane - etiology; Pemphigoid, Benign Mucous Membrane - metabolism; Pemphigoid, Benign Mucous Membrane - therapy; People with disabilities; Pharmacology; Phenotype; Review; Scars; Symptom Assessment; Tumor necrosis factor-TNF; Vascularization; Visual impairment; Autoimmune disease; Cytokines; Growth factors; Fibrosis; Ocular pemphigoid; Mucous membrane pemphigoid
• ISSN: 0257-277X; 1559-0755
• DOI: 10.1007/s12026-019-09087-7

Ocular mucous membrane pemphigoid (MMP) is a rare, immuno-mediated chronic progressive condition of the conjunctiva characterized by blisters developing from sub-epithelial tissue through disruption of the adhesions between the conjunctival epithelium and the sub-epithelium. Patients with ocular MMP, in many cases, develop profound conjunctival scarring and visual impairment. Furthermore, ocular MMP may lead to a progressive secondary corneal vascularization and to corneal opacification. Ocular MMP is difficult to diagnose during the initial stages because of false negatives during biopsy and variability in the clinical presentation. Most of the current pharmacological treatments aim to control the inflammatory response to reduce the progressive tissue remodeling which leads to the formation of a fibrotic scar. The course and prognosis of ocular MMP depend on the severity and progression of the disease after systemic immunomodulatory therapy. The aim of this review is to provide a comprehensive analysis of the current literature on established and emerging concepts in ocular MMP, with special attention to its clinical presentation, diagnosis, treatment, and pathogenic mechanisms, including the role of some cytokines and growth factors in the development of the disease.