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Acquired Hemochromatosis with Pronounced Pigment Deposition of the Upper Eyelids

机译:获得性血色素沉着症上眼睑色素沉着明显

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摘要

Hemochromatosis may be classified into two groups: primary (hereditary) or secondary (acquired). The acquired type most commonly occurs after massive intake of iron supplements or blood transfusions and is also known as transfusional iron overload. In the past, hemochromatosis was usually recognized at an advanced stage by the classic triad of hyperpigmentation, diabetes mellitus (“bronze diabetes”), and hepatic cirrhosis. Cutaneous hyperpigmentation is present in 70 percent of patients due to two different mechanisms: (1) hemosiderin deposition resulting in diffuse, slate-gray darkening and (2) increased production of melanin in the epidermis. A 47-year-old woman who receives regular transfusions due to low iron and chronic, unresolving anemia and who subsequently developed pronounced hyperpigmentation of the upper eyelids is described. The presentation, diagnosis, pathogenesis, and treatment options of hyperpigmentation due to secondary hemochromatosis are discussed.
机译:血色素沉着症可分为两类:原发性(遗传性)或继发性(获得性)。获得性类型最常见于大量摄入铁补充剂或输血后,也称为输血性铁超载。在过去,血色素沉着病通常在色素沉着的经典三联征,糖尿病(“青铜糖尿病”)和肝硬化中处于晚期。 70%的患者出现皮肤色素沉着过度,这是由于两种不同的机制:(1)铁血黄素沉积导致弥漫性,板岩灰色变暗和(2)表皮中黑色素的产生增加。描述了一名47岁的女性,该女性因铁水平低和慢性贫血而无法定期输血,随后出现明显的上眼睑色素沉着过度。讨论了由继发性血色素沉着症引起的色素沉着过度的表现,诊断,发病机制和治疗选择。

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