Scleroderma Renal Crisis-Like Acute Renal Failure Associated With Mucopolysaccharide Accumulation in Renal Vessels in a Patient With Scleromyxedema

Abstract

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New insights on scleromyxedema

“…An intrinsic abnormality of fibroblasts has never been identified, 8,14 while the quantification of the mucin deposits is not apparently related to the disease severity. 1,4,6–8,10–13 The demonstration that patients’ serum stimulates the fibroblasts proliferation and glycosaminoglycans synthesis in vitro supports the hypothesis of pathogenetic circulating cytokines. 15 Major promoters of glycosaminoglycans synthesis are interleukin (IL)-1, IL-6, tumor necrosis factor alpha and beta, as well as the transforming growth factor beta (TGF-b), 1 but no releasing source has been identified.…”

“…Extracutaneous manifestations in scleromyxedema are frequent, but a direct association with the pathogenic process was documented in very few cases, by the presence of mucin deposition in the internal organ biopsy or autopsy, 1,10–12 and probably they include unrelated comorbidities. 6 Normal thyroidal function is a diagnostic criterion, which exclude other forms of mucinosis.…”

“…1,[4][5][6][7][8] There are several systemic implications due to scleromyxedema, including renal, cardiac, pulmonary, and neurologic involvement, especially in the form of dermato-neuro syndrome. 1,[8][9][10][11][12][13] Unfortunately, the response to current treatments is not permanent, and the prognosis is further worsened by the sudden occurrence of hematological malignancies, and septic complications. 1,4,9 pathogenetic circulating cytokines.…”

Case 8-2015

“…The distribution of skin involvement in this patient is not typical of scleredema. [7][8][9] Many of the features described in this case could be explained by scleromyxedema; however, the absence of skin papules, one of the four diagnostic criteria, makes this diagnosis unlikely. Several cases of scleromyxedema have been associated with multiple myeloma.…”

European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis

“…Respiratory system: Dyspnoea, obstructive or restrictive pathology, hoarseness and aspiration due to decreased epiglottis and vocal cord mobility Kidneys: Acute renal failure is a rare event Eyes: Infrequent corneal opacities, ectropion. …”