Extracorporeal photochemotherapy: A potentially useful treatment for scleromyxedema

Brief communications Extracorporeal photochemotherapy: A potentially useful treatment for scleromyxedema Michael Berkson, MD, Gerald S. Lazarus, MD, Marie Uberti-Benz, MD, and Alain H. Rook, MD Philadelphia, Pennsylvania Scleromyxedema, a variant of papular mucinosis, is a rare condition that clinically resembles scleroderma. Itis characterized by a lichenoid papular eruption, induration of the skin, and a paraproteinemia of unknown significance. Histologic examination reveals dermal deposition of mucin; proliferation of large, stellate fibroblasts; and fibrosis. Multisystemic manifestations have also been reported. Scleromyxedema is often difficult to treat. One of the most effective therapies is melphalan or a similar alkylating agent but this carries the risk of acute toxicity and malignancy.l-4 We describe a patient who responded to extracorporeal photochemotherapy (photopheresis) after unsuccessful attempts with other treatment. Case report A 51-year-old black woman came for treatment in 1984 with skin tightening, thickening of the eyelids, a papular eruption, and monoclonal paraproteinemia. The clinical and histologic diagnosis was scleromyxedema. During the next 5 years she underwent treatment with several courses of plasmapheresis and PUVA. Each was discontinued because of poor tolerance or lack of response. She refused systemic chemotherapy. In November 1989, the patient was seen at The Hospital of the University of Pennsylvania with marked thickening of the skin of her face, neck, trunk, and upper extremities. She had infiltrates of the eyelids and periorbital area and numerous small papules primarily on the face, neck, and upper torso. Serum paraprotein (IgG-K) value was 1.3 gm/dl. She had no other systemic manifestations attributable to scleromyxedema. Monoclonal paraprotein in scleromyxedema suggests a state of immune dysregulation that may permit the clonal expansion of B lymphocytes within the peripheral blood. Moreover, because of the marked decrease in cutaneous sclerosis observed in the majority of photopheresis-treated scleroderma patients, and because photopheresis is well tolerated, this treatment was attempted. After informed consent, the patient began photopheresis on two consecutive days monthly. The patient improved with significant softening of her skin after 12 months oftherapy. She believes her skin is less tight and her neck is more supple. The papular eruption and the periorbital involvement have not progressed. Her paraprotein level From the General Clinical Research Center of the Hospital of the University of Pennsylvania, and the Department of Dermatology, Hospital of the University of Pennsylvania. Supported in part by grant M01RR00040-29 from the National Institutes of Health. Reprint requests: Alain H. Rook, MD, Department of Dermatology, Hospital of the University of Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104. 16/4/30376

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dropped from 1.3 to 1.1 gm/dl. She refused skin biopsy. She continues to undergo monthly photopheresis and tolerates the treatments well except for occasional nausea. Discussion. Gabriel et a1. 4 reviewed 11 scleromyxe-

dema patients treated with melphalan. Although two thirds showed regression of skin lesions, five patients had serious complications from therapy, including two cases ofleukemia and one oflymphoma. Photopheresis, the extracorporeal exposure of leukocytes to 8-methoxypsoralen and UVA light, is apparently safe and well tolerated. It is of proven benefit in advanced cutaneous T ceillymphoma and is currently undergoing trials in scleroderma. Thus far more than two thirds of patients demonstrate significant skin softening (Rook et at, manuscript in preparation) . Although the precise pathogenesis of sc1eromyxedema is unknown, it shares some features of scleroderma, particularly the increased dermal fibrosis. Our patient seemed to benefit from photopheresis, which, at present, appears not to have the risks associated with the alkylating agents. REFERENCES 1. Truhan AP, Roenigk HH Jr. The cutaneous mucinoses. J AM ACAD DERMATOL 1986;14:1-18. 2. Harris AO, Altman AR, Tschen JR, et al. Scleromyxedema. lnt J Dermatol 1989;28:661-7. 3. Harvey JM, Zilko Pl. Scleromyxedema with systemic involvement mimics rheumatic disease. Arthritis Rheum 1986;29:913-7. 4. Gabriel SE, Perry HO, Oleson GB, et al. Scleromyxedema: a scleroderma-like disorder with systemic manifestations. Medicine 1988;67:58-65.

AlbdofJDinalcylindrorna Michael S. Rabkin, MD, PhD,a and Susan D. Rollins, MOb Pittsburgh, Pennsylvania, and Johnson City, Tennessee Dermal cylindromas are characterized by cords and islands of small and large basophilic epithelial cells arranged in a "jigsaw-puzzle" configuration. The epithelial elements are surrounded by and interspersed with eosinophilic hyaline material that contains basement membrane components. Cystic and ductlike structures are commonly seen. More than 90% of cylindromas occur on the head and neck, sometimes in the setting of multiple cylindromas of the scalp ("turban tumors").l Extracranial cylindromas are rare, and most reported cases have been associated with multiple cranial cylindromas and/or have occurred in the context ofsyndromes From Rabkin Dermatopathology Laboratory, Pittsburgh,' and Outpatient Cytopathology Center, Johnson City.b Reprints not available. 16/4/30124