Thalidomide as a Potential Treatment for Scleromyxedema

Abstract

with a 1-year history of skin thickening. The process began on her hands and progressed to involve her arms, face, neck, shoulders, chest, and proximal extremities. Her only systemic complaints were limited mobility of her joints, particularly her hands and wrists. Physical examination findings revealed leonine facies. The skin of the neck, shoulders, and chest exhibited 1-to 2-mm coalescent linear papules. The skin of the hands, forearms, upper arms, and trunk was sclerotic. Skin biopsy results showed marked dermal thickening, displacement of collagen bundles by mucin, and numerous spindled fibroblasts, consistent with scleromyxedema. The results of laboratory tests, including a complete blood cell count, chemistry tests, liver function tests, an antinuclear antibody panel, an extractable nuclear antigen panel, and thyroid function tests, were normal. Immunoglobulins were obtained, and the following levels were revealed: IgM, 92 mg/dL (reference range, 25-210 mg/ dL); IgA, 152 mg/dL (reference range, 40-390 mg/dL); and IgG, 1490 mg/dL (reference range, 525-1650 mg/ dL), with a light chain monoclonal gammopathy. Hematologic evaluations, including serum and urine protein electrophoresis, immunoelectrophoresis, a bone marrow examination, and radiography of the head and chest, produced no evidence of multiple myeloma.Because of significant restriction of joint mobility, the patient underwent treatment with cytotoxic agents. She was initially treated with melphalan, 16 mg/d orally (PO), plus prednisone, 100 mg/d PO, for 4 days at a time in 1-month intervals for 4 cycles from

Scleromyxedema: Successful Treatment with Thalidomide in Two Patients

“…The efficiency of thalidomide in scleromyxedema has been described in 2 previous cases [1, 2]. We proposed thalidomide without any knowledge of these observations published after the introduction of the treatment in our 2 patients.…”

Successful Treatment of Scleromyxedema With Autologous Peripheral Blood Stem Cell Transplantation

“…Treatment options have included agents aimed at eradicating the plasma cell clone, such as chemotherapy, 3,[5][6][7][8] glucocorticoids, 9 and thalidomide, 10 as well as treatments aimed at blocking the paraneoplastic effects of the plasma cell clone, such as intravenous immunoglobulin, 11 extracorporeal photochemotherapy, 12 psoralen-UV-A, 13 elec-tron beam, 14 topical corticosteroids, interferon alfa, 15 and retinoids. Feasel et al 16 described a patient who achieved remission following high-dose chemotherapy with peripheral blood stem cell transplantation (PBSCT).…”

Scleroderma-like Fibrosing Disorders

“…Case reports cite variable improvement with other immunosuppressants including cyclophosphamide and cyclosporine (65)(66)(67)(68). There are multiple cases describing some benefit using thalidomide, although there remains a legitimate concern for the development of disabling peripheral neuropathy (68)(69)(70)(71)(72). More recent data note benefit of autologous stem cell transplantation in recalcitrant cases (53,(73)(74)(75).…”