Hemochromatosis-A Case Report-

血色素沉著病－一病例報告－

林建德(Chien-Te Lin) ； 張家綺(Cha-Chi Chang) ； 陳國熏(Gwo-Shing Chen)

《中華皮膚科醫學雜誌》 22卷4期 (2004/12) Pp. 333-337

摘要

血色素沉著病為一因慢性鐵質過多并沉積於組織而造成傷害的疾病。典型的症狀有肝硬化、皮膚呈青銅色到棕色之色素沉著、糖尿病及心肌病變。我們報告一46歲女性病患，主訴過去十年全身膚色漸進性地轉為青銅棕色，尤其以光照部位之色素沉著較為顯著。過去病史有糖尿病及慢性C型肝炎，但無輸血，服用或飲食攝取鐵劑。血液學檢查發現鐵質，鐵質蛋白及輸鐵蛋白飽和度皆為增加，影像學顯現有肝臟腫大現象。皮膚之組織病理學檢查顯示表皮基底層色素增加。鐵質染色呈現於血管旁，汗腺基底層及環繞其旁之結締組織有許多藍色顆粒沉積。肝臟切片之組織病理學顯示鐵質染色下有廣泛鐵質沉積之現象。此患者目前于門診接受放血、鐵螯合劑及胰島素注射，經過16個月治療後，其皮膚色素沉著已有淡化之現象。

並列摘要

Hemochromatosis is a disorder of chronic iron overload associated with tissue injury resulting from iron deposition. Typical findings include cirrhosis of liver, a bronze-brown pigmentation of the skin, diabetes mellitus, and cardiomyopathy. We describe a 46-year-old female patient presented with generalized bronze-brown pigmented skin with prominence over sun-exposed area for about 10 years. Diabetes mellitus and chronic hepatitis C history were traced, and she denied blood transfusion, medical or dietary iron ingestion. Increased serum iron and ferritin level accompanied with elevated transferrin saturation and hepatomegaly were investigated. The histopathologic study of skin showed hyperpigmentation in the basal layer of the epidermis. Iron stain revealed blue-staining granules mainly around blood vessels. More diffuse deposits of hemosiderin of liver tissue were also found. Lightening skin pigmentation was found after treatment with phlebotomy, chelating agent and regular insulin injection for 16 months. We herein present this case and review the literature.

並列關鍵字

Hemochromatosis ； Pigmentation ； Hemosiderin ； Iron stain