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红细胞生成性原卟啉病:你可能未曾见过它,但它可能已见过你。

Erythropoietic Protoporphyria: You May Not Have Seen It, but It May Have Seen You.

作者信息

Pastore Larissa M, Sun Christina W, Hsu Sylvia

机构信息

Dermatology, Cooper Medical School of Rowan University, Camden, USA.

Dermatology, Temple University Hospital, Philadelphia, USA.

出版信息

Cureus. 2022 Mar 17;14(3):e23253. doi: 10.7759/cureus.23253. eCollection 2022 Mar.

Abstract

Erythropoietic protoporphyria is a rare skin condition that commonly presents in childhood. We report a case of a 35-year-old Hispanic male with a history of sun sensitivity, presenting with complaints of immediate burning and itching of the skin on his face and upper extremities upon sun exposure. On examination, there was minimal face erythema and calluses over the knuckles. Laboratory workup demonstrated substantially increased protoporphyrin (over 10 times the upper limit of normal) along with elevated liver enzyme levels. Liver biopsy confirmed stage 4 cirrhosis. Our patient's cutaneous manifestations were the primary complaint that led to the diagnosis of his terminal hepatic illness. We recommend screening for erythropoietic protoporphyria in patients who present with a life-long history of non-blistering, burning and itching of the skin, which begins immediately upon sun exposure.

摘要

红细胞生成性原卟啉病是一种罕见的皮肤病,常见于儿童期。我们报告一例35岁的西班牙裔男性病例,该患者有日光敏感史,主诉在暴露于阳光下后,面部和上肢皮肤立即出现烧灼感和瘙痒。检查发现面部有轻微红斑,指关节处有胼胝。实验室检查显示原卟啉大幅增加(超过正常上限的10倍),同时肝酶水平升高。肝脏活检证实为4期肝硬化。我们患者的皮肤表现是导致其终末期肝病诊断的主要主诉。我们建议对有终生非水疱性、暴露于阳光下后立即出现烧灼感和瘙痒皮肤病史的患者进行红细胞生成性原卟啉病筛查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a02/9012550/1c88aace0196/cureus-0014-00000023253-i01.jpg

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