Untreated, scleromyxedema usually takes an unpredictable and potentially lethal progressive disease course over several years. A serious and potentially lethal complication is the dermato‐neuro syndrome which manifests with flu‐like prodromes followed by fever, convulsions and coma. Neurological, rheumatological, cardiovascular, gastrointestinal, respiratory tract, renal and ophthalmologic manifestations can occur, with decreasing frequency. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome‐shaped or flat‐topped, waxy, slightly red to skin‐colored papules and sclerodermoid indurations. Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. 2 5 14 in combination 0 0 3 0 17 Systemic corticosteroids 0 0 2 1 8 -in combination 0 0 2 3 20 Thalidomide 0 0 2 2 8 -in combination 0 0 2 0 10 Bortezomib -in combination 0 0 1 0 7 Stem cell transplant -in combination 0 0 1 2 9 Melphalan -in combination 0 0 2 1 10 Abbr.: RCT, randomized controlled trial PS, prospective study RS, retrospective study CS, case series CR, case report. There is no evidence of a causal relationship between paraproteinemia and disease manifestation.
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