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Cyclosporine is known to be effective in the treatment of psoriasis. In this study, we have used oral cyclosporine (6 mg/kg per day) given for 5 to 30 weeks to 24 patients for the treatment of 12 different dermatoses. Patients with the following diseases demonstrated a marked response or total clearing: 1 patient each with pyoderma gangrenosum, pityriasis lichenoides chronica, and psoriasis of the acrodermatitis continua of Hallopeau type. Moderate to marked response occurred in both patients with epidermolysis bullosa acquisita and the patient with hidradenitis suppurativa. Minimal to moderate responses were obtained in both patients with granuloma annulare, 1 of 2 with acrodermatitis continua of Hallopeau, both patients with Darier's disease, and 1 of 6 patients with vitiligo. Little or no response was noted in both patients with sarcoidosis, all 3 patients with pityriasis rubra pilaris, 5 of 6 patients with vitiligo, 1 patient with pemphigus foliaceous, and 1 with pemphigus vulgaris. Clinical side effects were mild and transient and included dysesthesia, fatigue, hypertrichosis, nausea, and flushing. The most frequent clinically significant abnormalities were hypertension and renal dysfunction, with all factors normalizing within 1 month of discontinuation of cyclosporine therapy.

We report the case of a 66-year-old man who presented with a spontaneous blistering involving both skin and mucous membranes, diagnosed as an inflammatory form of epidermolysis bullosa acquisita. During a 5-year course, the condition failed to respond to various therapies. Finally, only ciclosporin, together with a low dose regimen of prednisone, achieved a dramatic improvement with a total clearing within 6 months, persisting 1 1/2 years after discontinuation. Such results prompt us to propose such an association as a primary therapeutic scheme in an otherwise barely tractable disease.

Epidermolysis bullosa acquisita is a chronic, severe, subepidermal, blistering disease of the skin, characterized by marked resistance to topical and systemic therapy. This report concerns a well-documented case of a woman who had had epidermolysis bullosa acquisita for 6 years and had remained hospitalized continuously for 7 months in 1987. Her case ultimately was controlled with cyclosporine after the failure of a variety of therapeutic modalities in the hospital, including prednisone, methotrexate, azathioprine, phenytoin, vitamin E, gold sodium thiomalate (Myochrysine), isotretinoin, and plasmapheresis. In contrast to patients with pemphigus and pemphigoid treated with cyclosporine, our patient's autoantibodies did not disappear on therapy. Although its mechanism of action in epidermolysis bullosa acquisita is unknown, we propose that cyclosporine may be a helpful drug for patients whose disease is refractory to more traditional forms of therapy.