IVIG tedavisine yanıt veren bir monoklonal gammapatisiz skleromiksödem olgusu
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Dosyalar
Tarih
2012
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Liken miksödematozus deride fibroblast artışı ve müsin birikimi ile karakterize primer inflamatuvar bir dermatozdur. Skleromiksödem liken miksödematozusun jeneralize formu olup papüler lezyonlar, difüz skleroz ve eritem ile seyreder. Sıklıkla paraproteinemilerle birliktelik gösterir. Monoklonal gamopatisiz skleromiksödem ise liken miksödematozusun daha nadir görülen atipik bir varyantıdır. Sistemik tutulum da gösterebilen ve bazen fatal olabilen hastalığın tedavisinde olgu bildirileri şeklinde çok çeşitli seçenekler denense de henüz kesin bir tedavi şekli yoktur. İntravenöz immünglobulin ile yapılan olgu bildirilerinde ise yüz güldürücü sonuçlar elde edilmiştir. Skleromiksödemin etyopatogenezi tam olarak anlaşılamadığı gibi intravenöz immünglobulinin de skleromiksödemdeki etki mekanizması hâlen net değildir. Burada monoklonal gamopatinin eşlik etmediği bir skleromiksödem olgusu ve intravenöz immünglobulin tedavisine yanıtı sunulmaktadır.
Lichen myxedematosus is a primary inflammatory dermatosis characterized by fibroblast proliferation and mucin deposition in the skin. Scleromyxedema is the generalized variant of lichen myxedematosus and is accompanied by papular lesions, diffuse sclerosis and erythema. It is usually associated with paraproteinemias. Scleromyxedema without monoclonal gammopathy is a rare and atypical variant of lichen myxedematosus. Systemic involvement and sometimes fatal course can be seen with scleromyxedema. Although several treatment modalities have been tried, there is no definitive treatment available for the disease. Satisfactory results have been obtained in the case reports in which intravenous immunoglobulin treatment was used.The mechanism of action of intravenous immunoglobulin in scleromyxedema is still not clear as well as the etiopathogenesis of scleromyxedema. Herein, we describe the case of a patient with scleromyxedema without monoclonal gammopathy and his response to the treatment with intravenous immunoglobulin.
Lichen myxedematosus is a primary inflammatory dermatosis characterized by fibroblast proliferation and mucin deposition in the skin. Scleromyxedema is the generalized variant of lichen myxedematosus and is accompanied by papular lesions, diffuse sclerosis and erythema. It is usually associated with paraproteinemias. Scleromyxedema without monoclonal gammopathy is a rare and atypical variant of lichen myxedematosus. Systemic involvement and sometimes fatal course can be seen with scleromyxedema. Although several treatment modalities have been tried, there is no definitive treatment available for the disease. Satisfactory results have been obtained in the case reports in which intravenous immunoglobulin treatment was used.The mechanism of action of intravenous immunoglobulin in scleromyxedema is still not clear as well as the etiopathogenesis of scleromyxedema. Herein, we describe the case of a patient with scleromyxedema without monoclonal gammopathy and his response to the treatment with intravenous immunoglobulin.
Açıklama
Anahtar Kelimeler
Dermatoloji
Kaynak
Türkderm-Deri Hastalıkları ve Frengi Arşivi
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Cilt
46
Sayı
4
