Scleroderma-Like Illness as a Presenting Feature of Multiple Myeloma and Amyloidosis

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A 31-year-old woman with a history of bilateral carpal tunnel surgery complained of worsening hand pains and swelling. Subsequently, she presented for rheumatologic evaluation with generalized arthralgias, symmetric polyarthritis of the hands and feet, shiny skin with tightness and thickening, tender periungual erythema, malar rash, and photosensitivity. The only laboratory abnormality found then was a positive antinuclear antibody. Her joint symptoms were responsive to low-dose prednisone and hydroxychloroquine. However, the skin tightness progressed proximally and centrally and developed around the mouth. At that point, more specific autoimmune work-up showed negative relevant antibodies, and repeat antinuclear antibody tests turned out negative.

Later, she reported dysphagia and hoarseness, and ecchymotic rashes appeared on the face and forearm. Biopsy of the forearm lesion showed leukocytoclastic vasculitis. Staining for amyloid was negative. Subsequently, she was found to have hypogammaglobulinemia and Bence-Jones proteinuria; the progression of her skin symptoms provoked a repeat skin biopsy with special stains that demonstrated amyloidosis. Bone marrow biopsy showed >75% plasma cells, skeletal survey revealed multiple lytic lesions, and a diagnosis of multiple myeloma with associated amyloidosis was made. Despite the initial features of connective tissue disease in this young woman, a steadfast workup revealed the source of her problem.

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