Case report presents a 44-year-old man's scleromyxedema symptoms and treatment, highlighting high-dose intravenous immunoglobulin as a potential first-line therapy.
Scleromyxedema (SM) presents as a rare fibromucinous disorder, featuring generalized papular and sclerodermoid skin changes and monoclonal gammopathy, predominantly immunoglobulin G-lambda type. Middle-aged adults are primarily affected, with no gender or racial predilection. Beyond cutaneous manifestations, SM involves multiple organ systems, resembling systemic sclerosis, leading to significant morbidity and mortality. Dysphagia, proximal muscle weakness, and exertional dyspnea are prominent symptoms, with central nervous system involvement, such as encephalopathy and convulsions, being less common yet crucial. High-dose intravenous immunoglobulin has emerged as a potential first-line treatment. Here, we report the case of a 44-year-old man diagnosed with SM with paraproteinemia.
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Surani et al. (2025) studied this question.
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