A 53-year-old man presents with progressive induration of the skin and pruritus of the abdomen, upper chest, and arms of 3 months' duration (Figure 1). He also reports that pain and swelling of his hands developed after he handled halogenated hydrocarbons at work (Figure 2).
The patient has a history of hypertension and gastroesophageal reflux disease; he takes metoprolol, aspirin, and acetaminophen. Recently, he underwent surgery for bilateral carpal tunnel syndrome. The operation relieved the numbness in his hands.
Close examination of the skin reveals small, densely packed, waxy papules on the hands and forearms, abdomen, upper trunk, face, and neck. Other physical examination findings are normal.
The results of routine laboratory studies—including the erythrocyte sedimentation rate and an antinuclear antibody test—are normal. However, a small spike in the M protein level (0.2 g/dL) is noted on serum protein electrophoresis.
Biopsy specimens from the thickened skin on the right arm and abdomen reveal an increased number of fibroblasts in the papillary dermis. The increased cellularity and absence of markedly thickened collagen bundles rule out progressive systemic sclerosis (Figures 3 and 4). Increased mucin is noted; a colloidal iron stain highlights the increased acid mucopolysaccharide deposition (Figure 5). The cause is scleromyxedema.
Additional serologic studies reveal an underlying IgG γ paraproteinemia. A bone marrow biopsy specimen shows a slightly hypercellular marrow with scattered immature plasma cells; there is no evidence of underlying lymphoma or leukemia.
The patient subsequently becomes hoarse and dyspneic.Therapy with melphalan is started. After a few weeks, the pruritus, swelling, and "tightness" of his skin subside. The therapy also alleviates the pain and swelling of his hands as well as his dyspnea and hoarseness.
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