Scleroderma renal crisis-like acute renal failure associated with mucopolysaccharide accumulation in renal vessels in a patient with scleromyxedema.
ABSTRACT Scleromyxedema is a systemic disease characterized by lichenoid papules, nodules, and plaques on the skin and often diffuse skin induration resembling the cutaneous involvement of systemic sclerosis. The systemic involvement affects the musculoskeletal, pulmonary, cardiovascular, gastrointestinal, and central nervous systems, and the disorder is commonly associated with a paraproteinemia. Involvement of the kidney is rare and not considered a feature of the disease. Here, we describe an unusual case of scleromyxedema complicated by the development of scleroderma renal crisis-like acute renal failure with a marked intimal deposition of mucin, mucopolysaccharides, and hyaluronic acid in the intrarenal vessels.
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ABSTRACT: Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and that is associated with significant morbidity and mortality. Our purpose was to study the natural history of the disease and its response to therapy with alkylating agents. A clinicopathologic review of 26 patients with scleromyxedema was performed, and the extracutaneous findings and response to therapy with alkylating agents were noted. Extracutaneous manifestations, most often gastrointestinal, were present in 20 of 26 patients. An abnormal paraprotein was found in 23 of 26 patients, most commonly IgG-lambda (18 patients). Melphalan was used as therapy for 17 patients. The disease proved fatal in 10 of the treated patients. The overall prognosis in scleromyxedema is poor. Therapy is difficult. Although alkylating agents may prove beneficial in the short term, significant toxicity of these agents is apparent with long-term use.Journal of the American Academy of Dermatology 08/1995; 33(1):37-43. · 5.00 Impact Factor
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ABSTRACT: 15 renal dialysis patients have been identified with a skin condition characterised by thickening and hardening of the skin of the extremities and an increase in dermal fibroblast-like cells associated with collagen remodelling and mucin deposition. The disease closely resembles scleromyxoedema, yet has significant enough clinical and histopathological differences to warrant its designation as a new clinicopathological entity.The Lancet 10/2000; 356(9234):1000-1. · 39.21 Impact Factor
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ABSTRACT: Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.Journal of the American Academy of Dermatology 03/2001; 44(2):273-81. · 5.00 Impact Factor