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


Benign Mucosal Pemphigoid (Chronic Cicatricial Conjunctivitis; Cicatricial Pemphigoid; Essential Shrinkage of the Conjunctiva; Membrane Pemphigus; Ocularpemphigoid) 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 747b12h 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.

Bhol K, et al. Ocular cicatricial pemphigoid. A case report of monozygotic twins discordant for the disease. Arch Ophthalmol 1995; 113:202-207.

Kristensen EB, Norm MS. Benign mucous membrane pemphigoid, 1. Secretion of mucus and tears. Acta Ophthalmol

La Maza MS, et al. Cataract surgery in ocular cicatricial pemphigoid. Ophthalmology

Mondino BJ. Cicatricial pemphigoid and erythema multiforme. Ophthalmology 1990; 97:939.

Mondino BJ. Clinical immunologic disease. Dermatologic disease. In: Smolin G, Thoft RA, eds. The Cornea, 3rd ed. Boston: Little, Brown and Company, 1994:415-417.

Thomas CI, et al. Treatment of shrinkage diseases of the conjunctiva. Ann Ophthalmol 1974; 6:1289-l294.

Vandeveer MR. Ocular cicatricial pemphigoid. J Am Optom Assoc 1991; 62:166-l69.




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