- 期刊
Detection of Plasma Uroporphyrin Fluorescence as an Adjuvant Diagnostic and Follow-up Tool of Porphyria Cutanea Tarda
以血漿中尿紫質螢光檢測輔助診斷及追蹤遲發性皮膚紫質病
摘要
遲發性皮膚紫質病是由於尿紫質原脫羥基酶缺失所引發，引起此症主要的原因有c型肝炎、飲酒、雌性素、HIV感染、多氯碳氫化合物、藥物等諸多因素。本次報告的個案是位41歲男性，主訴有光敏感、雙手手掌背側水疱、光照部位色素沉著、以及多毛等症狀超過六個月。患者有酗酒習慣，而且有C型肝炎，此外，麩單酸轉氨酶／麩丙峒轉氨酶也升高。尿液Wood's lamp檢查呈珊瑚紅之陽性反應。病理切片結果顯示表皮下水疱，DIF在表皮真皮交界處及血管壁有IgA的線性沉積，IIF則為陰性反應。以spectrophotometer進行血漿螢光檢測結果顯示在398nm激發光時，可在619nm散射光下偵測到特異性吡咯紫質的吸收光譜。患者在接受多次放血以及hydroxychloroquine治療後症狀逐漸改善。在隨後的追蹤研究中則測量不到此特異螢光。利用此方法似乎可做為輔助診斷及追蹤PCT的工具。
關鍵字
無資料並列摘要
Porphyria cutanea tarda (PCT) is caused by the diminution of hepatic uroporphyrinogen decarboxylase activity. The clinical manifestation of PCT is associated mainly with hepatitis C, HIV infection, elevated iron, the use of alcohol, estrogen, polychlorinated hydrocarbons and certain drugs. We report a case of 41-year-old male who has been suffering from photosensitivity, blisters over bilateral dorsal hands, hyperpigmentation over sun-exposed areas and hypertrichosis for over six months. He is a victim of alcoholism and hepatitis C, with elevated aspartate aminotransferase (AST)/alanine aminotransferase (ALT). Wood’s lamp examination of urine showed positive finding with coral pinkish fluorescence. Histopathological examination of a skin lesion revealed subepidermal blister formation. Direct immunofluorescence (DIF) study indicated linear IgA deposition at dermo-epidermal junction and vessel walls, whereas indirect immunofluoroscence (IIF) exam was negative. Spectrophotometry study of the patient’s plasma in the acute stage by fluorescence spectrophotometer with excitation wavelength set at 398 nm, showed characteristic porphyria emission spectra at 619 nm. After multiple phlebotomy and oral hydroxychloroquine, his condition is gradually under control. No such specific fluorescence was observed in the following up studies. It seems that this method could be applied as an adjuvant diagnostic tool for PCT.