A 41 year-old man was referred to the National Institutes of Health (NIH) for evaluation of extensive skin thickening and rippled appearance of his upper extremities, torso, and lower extremities. The patient was day +671 status post a myeloablative 6/6 HLA-matched related (sister) peripheral blood stem cell transplant for acute myelogenous leukemia. The conditioning regimen consisted of busulfan and cyclophosphamide and graft-versus-host disease prophylaxis included tacrolimus and a short course of methotrexate.The patient's cutaneous symptoms began approximately eight months post-transplant with the onset of "red dots" on his skin accompanied by decreased range of motion at the wrists. A biopsy a patch on the right forearm demonstrated non-specific histologic findings of a mild superficial perivascular infiltrate with rare neutrophils. Prior to the NIH consultation, the patient had been treated with oral steroids, hydroxychloroquine sulfate, rituximab, thalidomide, and physical therapy without benefit. He had not been treated with phototherapy. Rather, the patient reported that the skin changes and range of motion at several joints continued to worsen, resulting in significant functional limitations. His immunosuppression regimen at the time of referral consisted of methylprednisolone 32 mg daily, tacrolimus 1.5 mg twice daily, hydroxychloroquine 200 mg twice daily, mycophenolate mofetil 1 g twice daily, and thalidomide 200 mg in the evening.
Physical ExaminationPhysical exam was remarkable for a widespread puckered, cellulite-like appearance of the bilateral inner upper arm, majority of the anterior torso, bilateral flanks, medial buttocks, and