Benign mucosal pemphigoid (chronic cicatricial conjunctivitis; cicatricial pemphigoid; essential shrinkage of the conjunctiva; membrane pemphigus; ocularpemphigoid) 137

General: Etiology unknown; involving older age group, especially over 70 years; chronic autoimmune disorder characterized by fibrosis beneath the conjunctival epithelium; associated with the major histocompatibility complex class I alleles, which confer susceptibility to the disease; likely due to a multigene effect and associated with environmental factors; incidence in women is twice as frequent as men, no geographic or racial predilection.

Ocular: Conjunctivitis; absence of goblet cells of conjunctiva; conjunctival ulcer; pannus and keratitis; corneal opacity; entropion; trichiasis; cicatrization of lacrimal ducts; corneal perforation; symblepharon; dry eyes; bilateral involvement (may be asymmetrical); ocular shrinkage; xerosis; conjunctival and corneal bullae.

Clinical: Subepidermal and subepithelial blistering of mucous membranes; blisters may occur in pharyngeal, laryngeal, nasal, anal, and genital mucosa.

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