nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное общество10.28996/2618-9801-2018-2-230-243nid-339Research ArticleНАБЛЮДЕНИЯ ИЗ ПРАКТИКИCASE REPORTSПоздняя кожная порфирия у пациентов на гемодиализе: литературный обзор и клинический случайPorphyria cutanea tarda in hemodialysis patients: review and a case reportАниконоваЛ. И.AnikonovaL. I.anikonovaspb@mail.ruРяснянскийВ. Ю.RyasnyanskiyV. Y.noemail@neicon.ruШосткаГ. Д.ShostkaG. D.noemail@neicon.ruПавленкоД. В.PavlenkoD. V.noemail@neicon.ruФГБУ ВО Северо-Западный медицинский университет им. И.И. МечниковаРоссияState Institute of higher education “Mechnikov North-Western State Medical University”Russian Federation201812082024202230243Copyright © Аниконова Л.И., Ряснянский В.Ю., Шостка Г.Д., Павленко Д.В., 20242024Аниконова Л.И., Ряснянский В.Ю., Шостка Г.Д., Павленко Д.В.Anikonova L.I., Ryasnyanskiy V.Y., Shostka G.D., Pavlenko D.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/339

Поздняя кожная порфирия (ПКП), наравне с псевдопорфирией, самый частый светочувствительный буллезный дерматоз у больных на гемодиализе. ПКП обусловлена нарушением синтеза гема в результате снижения активности печеночной уропорфириногендекарбоксилазы (УРО-Д). Расстройства в биосинтезе сопровождаются накоплением промежуточных метаболитов (порфиринов) в печени, плазме и коже. Основные проявления ПКП связаны с накоплением светочувствительных порфиринов в коже. У пациентов, получающих диализ, среди множества факторов, ассоциированных с развитием ПКП, наиболее актуальны вирусный гепатит С, перегрузка печени железом, подавляющие активность УРО-Д и нарушение клиренса порфиринов. Диагностика ПКП основана на характерной клинической картине и выявлении повышенного уровня порфиринов в плазме крови, а также в кале. Лечение направлено на исключение предрасполагающих факторов, уменьшение запасов железа и удаление порфиринов из печени и крови. Представлен случай ПКП у пациентки через 8,5 лет от начала хронического гемодиализа и перенёсшей ещё 2 обострения в течение последующих 7,5 лет. Взаимосвязь обострений с активностью вирусного гепатита С, показателями запасов железа прослеживалась не всегда. В связи с коморбидностью проведение терапевтических кровопусканий было проблематично. Для купирования кожных симптомов ПКП с успехом применялся гидроксихлорохин в сочетании с гемодиафильтрацией.

Porphyria cutanea tarda (PCT) and pseudoporphyria are most frequent photosensitive vesiculobullous skin disorder in hemodialysis patients. PCT is a disorder in the heme biosynthesis that results from a reduction in the activity of the hepatic enzyme uroporphyrinogen decarboxylase (URO-D). The abnormal heme biosynthesis produces a pathogenic accumulation of intermediary metabolites (porphyrins) in the liver, plasma and skin. The symptoms of PCT occur because of the accumulation of photosensitizing porphyrins in the skin. In patients receiving dialysis, PCT has several linked pathogenic mechanisms. The most relevant factors are viral hepatitis C, hepatic iron overload and impaired clearance of porphyrins. Diagnosis of PCT is established by identification of characteristic symptoms and finding a substantial elevation in the level of porphyrins in plasma; fecal porphyrins are also increased. Primary management of PCT involves removing susceptibility factors, depletion of iron stores, and reducing porphyrin levels in the liver and blood. We report a case of PCT that developed in a patient on maintenance HD for 8.5 years; 2 more exacerbations were observed in the next 7.5 years. The diagnosis of PCT was confirmed by the detection of a substantial elevation of porphyrins in plasma. The relationship between exacerbations with viral hepatitis C activity, iron levels was not always observed. Because of comorbidity, therapeutic phlebotomy was problematic and treatment modality was combination of hydroxychloroquine and haemodiafiltration.

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The authors declare that there are no conflicts of interest present.