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- W2170873524 abstract "Scleromyxedema is a rare connective tissue disorder, characterized by generalized skin induration with waxy yellow–red papules. Histological features include cutaneous mucin deposition in the superficial dermis, specifically hyaluronic acid, and fibroblast proliferation. Most patients with this disorder present with monoclonal paraproteinaemia, mainly IgG lambda. Oesophageal abnormalities with dysphagia, muscle weakness, dyspnoea with abnormal diffusing capacity, restrictive lung disease and cor pulmonale, pericardial effusions, central nervous system abnormalities and neuropathies, arthralgias and Raynaud’s phenomenon have been observed, and may be associated with significant morbidity and mortality [1–3]. In postmortem studies, interstitial and vascular mucin deposition in inner organs has been found inconsistently [2,4]. Renal disease is rare in patients with scleromyxedema. In this article, we report a middle-aged female with scleromyxedema and a scleroderma-like acute renal crisis." @default.
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- W2170873524 date "2007-04-20" @default.
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- W2170873524 title "Scleroderma-like acute renal crisis in a patient with scleromyxedema" @default.
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- W2170873524 doi "https://doi.org/10.1093/ndt/gfm103" @default.
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