B. G. TÜRK, B. ERTEKİN, A. Ö. SEXGİN, A. C. KAZANDI, T. DERELİ, F. ÖZDEMİR

Skleromiksödem tedavisinde sistemik steroidin hızlı etkinliği

Skleromiksödem dermiste müsin birikimi, fibrozis ve monoklonal gammopati ile karakterize nadir görülen, kronik seyirli, nedeni bilinmeyen bir hastalıktır. Diğer deri müsinozlarından dermiste müsin birikimi yanısıra fibrozis görülmesi, jeneralize papüler ve sklerodermoid erüpsiyon, monoklonal gammopati varlığı ve tiroid hastalığının eşlik etmemesi ile ayrılır. Deri dışında diğer organ sistemleri de tutulabilmektedir. Skleromiksödem tedavisi oldukça güç bir hastalıktır. Görülme sıklığı nedeniyle bu hastalıktaki tedavi deneyimi olgu bildirileri ve serileri ile sınırlıdır. Bu nedenle burada sistemik kortikosteroid tedavisine dramatik yanıt gösteren skleromiksödemli bir olgu sunulmaktadır.

Rapid response to systemic steroids in the treatment of scleromxyedema

Scleromyxedema is a chronic, rare disease with an unknown etiology which is characterized by fibrosis and mucin deposition in dermis and monoclonal gammopathy. Scleromyxedema is differentiated from the other cutaneous mucinoses with the presence of mucin deposition with fibrosis, generalized papular and sclerodermoid eruption, monoclonal gammopathy and with the absence of a tiroid disorder. The other organ systems may also be involved. The treatment of the disease is particularly difficult. Because of the rarity of the disease, experience with the treatment is limited to case reports and series. Therefore herein, a dramatic resolution of scleromyxedema with systemic streoid treatment is presented.

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