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一例与象皮病相关的经典疣状表皮发育不良:一种非典型表现。

A case of classic epidermodysplasia verruciformis associated with elephantiasis: an atypical presentation.

作者信息

Boutaarourt Meryeme, El Jouari Ouiame, Gallouj Salim

机构信息

Department of Dermatology-Venereology, Mohamed VI University Hospital, M3MF+GCG, 90100, La Nouvelle Ville Ibn Batouta, Tangier, Morocco.

Faculty of Medicine and Pharmacy, M3MF+GCG, 90100, La Nouvelle Ville Ibn Batouta, Tangier, Morocco.

出版信息

Oxf Med Case Reports. 2025 Jul 14;2025(7):omaf104. doi: 10.1093/omcr/omaf104. eCollection 2025 Jul.

Abstract

Epidermodysplasia verruciformis (EV) is a rare genetic skin disorder characterized by increased susceptibility to human papillomavirus infections (HPV) and a high risk of cutaneous carcinomas. We report the case of a 30-year-old female patient presenting with diffuse verrucous lesions, predominantly affecting the extremities, associated with unilateral elephantiasis. The diagnosis was histologically confirmed, revealing vulgar warts consistent with EV. HIV serology was negative, and systemic retinoid therapy was initiated. EV is linked to mutations in the EVER1 and EVER2 genes, predisposing patients to chronic infections with specific HPV types. Close monitoring is crucial to prevent malignant transformation. This case highlights the diagnostic and therapeutic challenges of EV, emphasizing the importance of an appropriate management strategy to control lesions and prevent complications.

摘要

疣状表皮发育不良(EV)是一种罕见的遗传性皮肤病,其特征是对人乳头瘤病毒(HPV)感染的易感性增加以及皮肤癌的高风险。我们报告了一例30岁女性患者,其表现为弥漫性疣状病变,主要累及四肢,并伴有单侧象皮肿。组织学检查确诊为EV,可见与疣状表皮发育不良相符的寻常疣。HIV血清学检查为阴性,并开始进行系统性维甲酸治疗。EV与EVER1和EVER2基因的突变有关,使患者易患特定HPV类型的慢性感染。密切监测对于预防恶性转化至关重要。该病例突出了EV的诊断和治疗挑战,强调了采取适当管理策略以控制病变和预防并发症的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d03/12258038/a55a8d1ebbf1/omaf104f1.jpg

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