氨苯砜与肝铁质沉着病(法语)

来源 :世界核心医学期刊文摘(皮肤病学分册) | 被引量 : 0次 | 上传用户:sukeyjuan
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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 of C282Y mutation of the HFE gene. Case n__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 serum ferritin and free iron with hemolysis were found. A diagnosis of genetic hemochromatosis was ruled out by the absence of C282Y mutation of the HFE gene. Case The absence of C282Y mutation (HFE gene) -ruled out of a diagnosis of In our two cases, hepatosiderosis was noted after long-term administration of Disulone ™. 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 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. The cases raise the question of the need for serum ferritin analysis during Disulone ™ therapy.
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