P39 Treatment of scleromyxedema with intravenous immunoglobulin
نویسندگان
چکیده
منابع مشابه
Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin.
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...
متن کاملManaging scleromyxedema with intravenous immunoglobulin: acute worsening of scleromyxedema with biclonal gammopathy.
Scleromyxedema is a rare chronic cutaneous mucinosis usually associated with a monoclonal gammopathy and underlying systemic disease. The etiology of the disease is not known. There are no standard treatments and response to various therapeutic modalities varies. We report a case of refractory scleromyxedema in a 63-year-old man with a biclonal IgG and IgM λ-gammopathy. The patient was successf...
متن کاملScleromyxedema with neurologic involvement: Therapy with intravenous immunoglobulin
Umbilical cord blood transplantation can cure pruritic eczematous eruption in a hypohidrotic ectodermal dysplasia with immunodeficiency patient Satoko Minakawa, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan; Daisuke Sawamura, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan...
متن کاملScleromyxedema with Subcutaneous Nodules: Successful Treatment with Thalidomide and Intravenous Immunoglobulin
Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg) appears to be the therapy of choice for scleromyxedema. Treatment ...
متن کاملScleromyxedema: a case series highlighting long-term outcomes of treatment with intravenous immunoglobulin (IVIG).
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...
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ژورنال
عنوان ژورنال: Rheumatology
سال: 2020
ISSN: 1462-0324,1462-0332
DOI: 10.1093/rheumatology/keaa111.038