conclusions this study indicated that the rv systolic strain and strain rate can be used to detect early rv systolic dysfunction in ssc patients without pulmonary hypertension. these parameters may be useful for the provision of a more adequate management of ssc patients. results in the ssc patients, the rv strain (- 19 ± 10 vs. - 25 ± 4 %; p = 0.004) and the systolic strain rate (- 1.3 ± 0.5 v...

1. Rongioletti F. Lichen myxedematosus (papular mucinosis): New concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100‐4. 2. Mateos M, Hernández M, Giraldo P, Rubia J, Arriba F, Corral L, et al. Lenalidomide plus Dexamethasone for High‐Risk Smoldering Multiple Myeloma. N Engl J Med 2013;369:438‐47. 3. Brunet‐Possenti F, Hermine O, Marinho E, Crickx B, Descamps V. Combina...

Scleromyxedema is a rare disease characterized by extensive mucin deposition with fibrosis, and is associated with a monoclonal gammopathy. Currently there is no consensus on optimal treatment of this potentially fatal disease because of the lack of randomized controlled trials and limited number of case reports. At the time of this writing, 24 cases were published reporting clinical improvemen...

P ARAPROTEINS* ARE generally found in the serum in patients suffering from multiple myeloma or Waldenstr#{246}m’s macroglobulinemia. Recently, however, there have been a number of reports on patients in whom paraproteinemia#{176} was found in association with a myeloproliferative disorder such as polycythemia, erythroleukemia, acute myeloblastic and lymphoblastic leukemia, and chronic myelocyti...

CORRESPONDENCE Scleromyxedema diagnosis following unexplained encephalopathy Scleromyxedema is a rare idiopathic disorder, characterized by papules in a thickened mucinous dermis, with fi bro-blastic proliferation and monoclonal paraproteinemia [1, 2]. Typically, scleromyxedema affects middle-aged men, has a chronic progressive course and frequently pre sents extracutaneous complications that g...

Although scleromyxedema has been associated with neoplasm in rare instances, the literature showed no evidence of association with seminoma. We report a 43-year-old man who presented with a scleromyxedema and relapsed seminoma. The skin lesions of scleromyxedema cleared completely on treatment of seminoma with chemotherapy.

Scleromyxedema is a rare disease characterized by mucin deposition in skin and other organs and the production of a monoclonal IgG protein. Herein we describe our experience with a series of patients with this condition and specifically focus on the use of intravenous immunoglobulin (IVIG) for long-term management. We retrospectively reviewed the clinical manifestations of 10 patients evaluated...

Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It defined by generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment known those tried have inconsistent results. The first-line intravenous immunoglobulin infusions variable response frequent relapse. We describe, here, patient scleromyxedema lambda gammopat...

a 63-year-old woman suffering from progressive systemic sclerosis for about 20 years disclosed symptoms of liver disease within the last three years. diagnosis of biliary cirrhosis was established on the basis of clinical picture, pathological examination of the hepatic tissue sample, immunological tests, and x-ray studies. association of systemic sclerosis with primary biliary cirrhosis is bri...