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假卟啉症

Author
Craig A Elmets, MD
Section Editor
Maja Mockenhaupt, MD, PhD
Deputy Editor
Rosamaria Corona, MD, DSc
Translators
林麟, 主任医师

引言

假卟啉症是一种大疱性光照性皮肤病，具有迟发性皮肤卟啉病(porphyria cutanea tarda, PCT)的临床和组织学特点，但卟啉代谢无异常[1]。假卟啉症可能与药物，慢性肾衰竭和血液透析，以及使用晒黑床有关。假卟啉症的典型表现为手背部或其他曝光部位皮肤脆性增加，出现水疱，粟丘疹，以及瘢痕形成。从组织学角度来看，存在非炎性表皮下水疱。

本专题将讨论假卟啉症的发病机制、临床表现、诊断和治疗。PCT以及与光敏感性有关的其他类型卟啉症将单独讨论。 (参见“迟发性皮肤卟啉病和肝红细胞生成性卟啉病”和“变异性卟啉病”和“先天性红细胞生成性卟啉病”)

流行病学

假卟啉症的发病率尚不清楚。它可以在任何年龄发病，女性比男性多见。已报道约有10%的使用萘普生的儿童发生了假卟啉症，萘普生是一种常规性用于治疗幼年特发性关节炎的非甾体类抗炎药(nonsteroidal antiinflammatory drug, NSAID)。在使用萘普生治疗的儿童中，发生假卟啉症的危险因素包括皮肤光分型为Ⅰ型或Ⅱ型(表 1)，眼睛呈蓝色/灰色，以及同时使用氯喹[2-4]。

病因和发病机制

已有人提出4项与假卟啉症的病因相关的因素：紫外线(ultraviolet, UV)辐射，药物，慢性肾衰竭和血液透析，以及晒黑床。

紫外线辐射 — 虽然确切的发病机制尚不清楚，但是药物诱导的假卟啉症被认为是一种光动力性光毒性药物反应。在这个类型的反应过程中，光敏化合物在吸收适当波长的光辐射后变成活化状态并与氧起反应形成活性氧类(reactive oxygen species, ROS)，如单态氧、过氧化氢或超氧阴离子。ROS可以破坏细胞成分，如核酸、细胞膜、脂质和蛋白质。炎症介质和炎症细胞均参与组织损伤，包括补体激活的产物、促炎症反应细胞因子、花生四烯酸代谢物、蛋白酶以及多形核白细胞。

              

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Literature review current through: 2017-06 . | This topic last updated: 2017-07-12.
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