Eosinophilic fasciitis responsive to treatment with pulsed steroids and cyclosporine

A 58-year-old white woman presented with a 7-month history of indurated hyp erpigmented plaques on her upper back, chest, abdomen, and arms. On the for earms and shins, associated with the induration, there was a waxy and shiny appearance. The lesions affected the dorsal aspect of both hands and feet, but spared the fingers and toes (Fig. 1). Gradually, as her skin became mo re indurated, she developed flexural contractures, limiting her range of mo vement and impairing her daily physical activities. She denied other system ic symptoms. She was empirically begun on prednisone, 20 mg daily, with a presumptive di agnosis of eosinophilic fasciitis; however, she did not respond to this the rapy. At this point, laboratory examination revealed a normal complete bloo d count, and negative antinuclear antibodies and rheumatoid factor. The bio psy specimen showed a sparse perivascular lymphocytic infiltrate without eo sinophils. The deep reticular dermis was thickened, and edematous, homogeni zed, horizontally oriented collagen bundles extended down fibrous septa int o the fat. The underlying fascia was massively thickened and sclerotic with focal lymphoplasmacytic inflammatory infiltrate extending to the muscle (F igs 2 and 3). The patient then received a course of pulsed methylprednisolone, 1 g daily, for 5 days, and was started simultaneously on cyclosporine, 150 mg, twice a day. Within 3 weeks, she had significant reduction of the skin induration as well as improved range of motion of her joints.