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Ocular mucous membrane pemphigoid presents some of the most challenging dilemmas in anterior segment management. Diagnosis is made difficult by the insensitivity of immunopathological investigations and the differential diagnosis of other scarring conjunctival disorders. The management of the associated ocular surface disease involves control of blepharitis, dry eye, filamentary keratitis, keratinization, lid malposition, and persistent epithelial defect, as well as the identification and avoidance of toxicity. Inflammation associated with the underlying disorder demands the use of systemic immunosuppressive therapy in many patients. New biological immunotherapies have been used when conventional immunosuppressive therapies fail. Ophthalmic plastic surgery is essential for the management of lid malposition and corneal exposure. Improving vision may require the use of specialized contact lenses, cataract surgery, and corneal and ocular surface reconstructive surgery. All surgery must be integrated with ocular surface treatment and immunosuppressive treatment to avoid disease exacerbations.

We report the case of a 73-year-old Japanese woman suffering from antiepiligrin (laminin 5) cicatricial pemphigoid (CP) with typical clinical and immunopathological features. Histologically, the lesional mucous membrane showed a subepidermal blister formation. When indirect immunofluorescence techniques with skin split by 1 M NaCl as the substrate were used, the patient's serum reacted only to the dermal side. Immunoprecipitation studies demonstrated that the patient's serum contained IgG autoantibodies directed against a set of polypeptides that corresponded to epiligrin (laminin 5). After corticosteroids and immunosuppressive agents had been administered systemically, the patient's autoantibody titer decreased and the cutaneous and mucosal blister formations were suppressed. However, the ocular lesions persisted in spite of these therapeutic regimens. After combining these treatments with double-filtration plasmapheresis, the ocular lesions improved and showed almost no progression. Plasmapheresis may thus present a new option for the treatment of CP., (Copyright 2000 S. Karger AG, Basel.)

Cicatricial pemphigoid is a systemic autoimmune disease with both ocular and extraocular manifestations. Its effect on the eyes is characterized by chronic cicatrizing conjunctivitis and progressive conjunctival sub-epithelial fibrosis with resultant fornix foreshortening, symblepharon formation, trichiasis, meibomian, and lacrimal duct obstruction resulting in sicca syndrome, ocular surface keratinization, and eventually blindness. Extraocular manifestations include stricture formation of the esophagus, trachea, anus, or vagina. OCP is characterized by deposition of immunoreactants at the conjunctival basement membrane zone, initiating a type II hypersensitivity reaction. Demonstration of this deposition can be done using immunofluorescence or immunoperoxidase technique.

Cicatricial pemphigoid (CP) and erythema multiforme (EM) are bullous diseases that involve the skin and mucous membranes including the conjunctiva. Of all the bullous diseases, CP and EM not only involve the conjunctiva most frequently but also cause the most severe conjunctival disease. A chronic, progressive disease, CP is characterized by shrinkage of the conjunctiva, symblepharon, entropion, trichiasis, dry eye, and finally reduced vision from corneal opacification. It is primarily a disease of the elderly that affects more women than men and is characterized by blisters or bullae in a subepithelial location and immunoglobulins and complement bound to the basement membrane zone of skin and mucous membranes including the conjunctiva. Circulating antibodies to the basement membrane zone can be demonstrated occasionally. Treatment includes artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, and systemic immunosuppressive therapy including corticosteroids. An acute, generally self-limited, inflammatory disorder of the skin and mucous membranes, EM occurs primarily in young, healthy individuals. The most frequent precipitating factors are (1) drugs and (2) infections caused by Mycoplasma pneumoniae and herpes simplex. Conjunctival involvement ranges from a mild catarrhal conjunctivitis which terminates without sequelae to membranous conjunctivitis which may heal leaving scarring, symblepharon, and even ankyloblepharon. Histopathologic findings include subepithelial bullae and perivascular mononuclear cell infiltrates. Patients with EM have circulating immune complexes and immunoreactant deposition in the blood vessel walls of the dermis. After the acute episode has subsided, they may require artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, tarsorrhaphy, and mucous membrane grafts.

The characteristic feature of ocular cicatricial pemphigoid (OCP) is progressive shrinkage of the conjunctiva. In our series of 78 patients with OCP, 21% had cutaneous involvement and 50% had involvement of the oral mucosa. Immunoglobulins and the third component of complement are found bound to the conjuctival epithelium and basement membrane of patients with OCP. Circulating antibodies which bind to the conjunctival and corneal epithelium but not to the conjunctival basement membrane have also been demonstrated. OCP is associated with an increased prevalence of HLA-B12. The lids and conjunctiva of patients with OCP demonstrate an increased incidence of potential pathogens when compared with age- and sex-matched controls. When followed for a period averaging 22 months, the majority of patients not treated with systemic immunosuppressives or topical corticosteroids progress. However, OCP has a variable course because there were patients in all stages who did not progress. The acute manifestations of OCP may cause rapid shrinkage of the conjunctiva and may be suppressed systemic corticosteroids.