Scleromyxedema with neurologic involvement: Therapy with intravenous immunoglobulin
نویسندگان
چکیده
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; Eturou Ito, MD, PhD, Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan; Hajime Nakano, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, HIrosaki, Aomori, Japan; Hajime Takeda, MD, PhD, Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan
منابع مشابه
Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema.
Scleromyxedema is a rare, generalized form of lichen myxedematosus, which may be associated with systemic involvement and can be fatal. The therapeutic options available provide partial or inconsistent response and are associated with significant adverse effects. We report a case of scleromyxedema with cardiac involvement, treated with low-dose intravenous immunoglobulin, with almost complete c...
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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...
متن کامل[Good response of scleromyxedema and dermato-neuro syndrome to treatment with intravenous immunoglobulins].
Scleromyxedema is a potentially serious disease that can have various systemic complications. One of the most frequent forms of central nervous system involvement is dermato-neuro syndrome. High-dose intravenous immunoglobulins are among the drug treatments that have been used for this syndrome. We describe 2 patients with scleromyxedema, one of whom developed dermato-neuro syndrome. Both patie...
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Background: Children with idiopathic thrombocytopenic purpura who are treated with intravenous immunoglobulin therapy might experience a decline in their absolute neutrophil count. The aim of this study was to investigate the incidence of neutropenia following intravenous immunoglobulin therapy in children with idiopathic thrombocytopenic purpura undergoing intravenous immunoglobulin therapy. ...
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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...
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