長期血液透析患者に合併した晩発性皮膚ポルフィリン症におけるポルフィリン代謝異常の証明と治療

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  • Porphyrin metabolism abnormalities and treatment in a uremic patient with porphyria cutanea tarda.

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A thirty-seven-year-old female uremic patient who had been on maintenance hemodialysis for seven years developed bullous dermatosis, pigmentation, minor scarring and skin fragility on the face, forearms and hands. The skin biopsy suggested characteristic findings of porphyria cutanea tarda (PCT). In this patient and four other agematched female uremic patients without PCT-like dermatosis, all fecal and dialysate porphyrin analyse were performed. The patient showed high amounts of fecal uroporphyrin (URO), 559μg/24h (15.8±5.3 in control group), coproporphyrin (COPRO), 914μg/24h (227.8±65.6), and heptacarboxyporphyrin (HEPTA), 1, 785μg/24h (22.0±14.7). However, there was little difference in protoporphyrin level as compared to controls (267 vs 233.5±114.7 in μg/24h). Both the URO/COPRO and the HEPTA/COPRO ratio in the patient were higher than those in the control group. The concentrations of URO and COPRO in dialysate were 8μg/l and 4μg/l one hour after the beginning of hemodialysis and 18μg/l and 2μg/l three hours after, respectively. Those in the control group were 3.3±0.6 and 1.3±0.3 (one hour after hemodialysis) and 3.5±1.2 and 1.3±0.3 (after three hours), respectively. These results confirmed that the patient had PCT. Aluminum hydroxide gel and ferrous sulphate supplementation were discontinued. Minimizing the exposure of skin, dialyzer and hemodialysis tubing to sunlight reduced the bullous lesions and erosion.

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