氨苯砜与肝铁质沉着病(法语)  

Disulone and hepatosiderosis (Fren)

作　　者：Darrieux L. Adamski H Turlin B. 王琼 

机构地区：[1]Service de Dermatologie, CHU Pontchaillou, rue H. Le Guilloux, 35033 Rennes Cedex

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第12期54-54,共1页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Background. DisuloneTM （dapsone +iron oxalate） is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of DisuloneTM. Patients and methods. Case n<sub>1. A 51-year-old manwas treated with DisuloneTM for a neutrophilic skin disease. After 17 years of treatment, elevated serumferritin and free iron with hemolysis were found. Liver biopsy confirmed hepatosiderosis. A diagnosis of genetic hemochromatosis was ruled out by the absence of C282Y mutation of the HFE gene. Case n<sub>2. A 52-year-old man receiving DisuloneTM for dermatitis herpetiformis for 25 years presented elevated serumferritin and free iron with hemolysis. Hepatic iron overload was confirmed by liver biopsy. The absence of C282Y mutation （HFE gene）-ruled out a diagnosis of genetic hemochromatosis. Discussion. In our two cases, hepatosiderosis was noted after long-term administration of DisuloneTM. This complication has been reported only rarely. In murine models, a relationship was found between prolonged administration of dapsone and hepatic iron overload as revealed by hemolysis. Although it is difficult to extrapolate this relationship to humans with any certainty, our patients had also chronic hemolysis and iron overload secondary to administration of DisuloneTM. Moreover in France, dapsone is marketed in combination with iron oxalate, with the attendant risk of iron overload. These cases raise the question of the need for serum ferritin analysis during DisuloneTM therapy.Background. DisuloneTM (dapsone +iron oxalate) is a sulfone used in the treatment of numerous skin diseases. We report two cases of hepatosiderosis secondary to long-term administration of DisuloneTM. Patients and methods. Case n__1. A 51-year-old manwas treated with DisuloneTM for a neutrophilic skin disease. After 17 years of treatment, elevated serumferritin and free iron with hemolysis were found. Liver biopsy confirmed hepatosiderosis. A diagnosis of genetic hemochromatosis was ruled out by the absence...

关 键 词：氨苯砜 TM 患者 血清铁蛋白 病例 苯胺 铁超负荷 遗传性血色素沉着病 

分 类 号：R575[医药卫生—消化系统]