A 58-year-old white woman presented with a 7-month history of indurated hyperpigmented plaques on her upper back, chest, abdomen, and arms. On the forearms 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 more indurated, she developed flexural contractures, limiting her range of movement and impairing her daily physical activities. She denied other systemic symptoms.
She was empirically begun on prednisone, 20 mg daily, with a presumptive diagnosis of eosinophilic fasciitis; however, she did not respond to this therapy. At this point, laboratory examination revealed a normal complete blood count, and negative antinuclear antibodies and rheumatoid factor. The biopsy specimen showed a sparse perivascular lymphocytic infiltrate without eosinophils. The deep reticular dermis was thickened, and edematous, homogenized, horizontally oriented collagen bundles extended down fibrous septa into the fat. The underlying fascia was massively thickened and sclerotic with focal lymphoplasmacytic inflammatory infiltrate extending to the muscle (Figs 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.