Scleromyxedema associated with scleroderma renal disease and acute psychosis

Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin

“…13 It is a rare manifestation of scleromyxedema with only 10 documented cases. [6][7][8]12,13,[39][40][41][42][43] Typically, dermatoneuro syndrome has been treated with intensive care monitoring, [39][40][41] plasmapheresis, 42 IV steroids, 8,12 or combinations of these treatments with variable outcomes. 13,43 Improved mental status has been observed in patients treated with plasmapheresis alone 42 and patients given combined treatment with both plasmapheresis and steroids.…”

Scleromyxoedema and thrombotic thrombocytopaenic purpura: two rare conditions both responding to plasma exchange

“…Some authors believe it is a benign condition limited to the skin with a chronic course [3] and of cosmetic significance only. Well documented complications include proximal myopathy [5,6] which is often inflammatory, carpal tunnel syndrome [2], dysphagia [2,5], psychosis [5,7] and seizures [ 5 ] . However, this is a rare event and most believe that generalised scleromyxoedema carries a poor prognosis.…”

“…Spontaneous regression has even been described [4]. Other associations include pulmonary fibrosis [2], cardiomyopathy and scleroderma-like renal disease [7]. In a clinicopathological review of 26 patients with scleromyxoedema [2], 20 had extracutaneous disease.…”

Cutaneous mucinoses

“…120 An unusual granulomatous variant of scleromyxedema has been reported. 80,81 Bizarre neurological symptoms, 82 underlying carcinoma, 56,83,84 chronic hepatitis C, 85 pachydermoperiostosis, 56 dermatomyositis, 86 scleroderma, 81,87 atherosclerosis, 88 esophageal aperistalsis, 89 and multiple keratoacanthomas 78 have all been reported in association with scleromyxedema. 121 The changes in nephrogenic systemic fibrosis are similar to those seen in scleromyxedema (see below).…”

“…There were interstitial granuloma annulare-like features. 81 Scleromyxedema is usually progressive, but spontaneous resolution has been reported. Involvement of the subcutis is reported.…”