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Complete Remission of Scleromyxedema Following Autologous Stem Cell Transplantation
Adrienne M. Feasel, MD;
Michele L. Donato, MD;
Madeleine Duvic, MD
Arch Dermatol. 2001;137:1071-1072.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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INTRODUCTION
Scleromyxedema is characterized by dermal fibroblast proliferation and mucin deposition, associated with plasma cell dyscrasia. Therapy for systemic progression is often not effective, and the disease is potentially fatal.1 We describe a man with rapidly progressive scleromyxedema in whom multiple treatments had failed before complete remission was achieved with treatment with high-dose pulse dexamethasone, high-dose melphalan, and autologous stem cell transplantation.
REPORT OF A CASE
A 46-year-old white man was referred to the M. D. Anderson Cancer Center, Houston, Tex, to undergo photopheresis for scleromyxedema. Six years earlier, pruritic papules appeared on the dorsal part of his hands but resolved with oral and topical corticosteroid therapy. Three years later, the lesions returned as waxy plaques, thickened skin, and orbital swelling. A skin biopsy specimen showed dermal mucinosis and fibrosis, consistent with scleromyxedema. IgG light chain plasma cell dyscrasia was noted on serum electrophoresis. . . . [Full Text of this Article]
COMMENT
From the Departments of Dermatology (Drs Feasel and Duvic) and Bone Marrow Transplantation (Dr Donato), The University of Texas, M. D. Anderson Cancer Center, Houston.
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