Back issues No.4 - 2001 / Case Report  

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Detection of Plasma Uroporphyrin Fluorescence as An Adjuvant Diagnostic and Follow-up Tool of Porphyria Cutanea Tarda
Yuan-Yue Hou, Roger Chin-Che Hsu, Ming-Tsan Wang, Jwu-Lai Yeh, Hsin-Su Yu
Dermatol Sinica 19: 316-323, 2001

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.

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