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[一名疣状表皮发育不良伴多发鳞状细胞癌患者接受低剂量预防性口服异维甲酸治疗18年]

[Low-dose prophylactic oral isotretinoin treatment for 18 years in a patient with epidermodysplasia verruciformis and numerous squamous cell carcinomas].

作者信息

Troyanova-Slavkova Svetoslava, Eickenscheidt Lena, Pönnighaus Jörg-Martin, Kowalzick Lutz

机构信息

Klinik für Hautkrankheiten und Allergologie, HELIOS Vogtland-Klinikum Plauen GmbH, Postfach 100153, 08505, Plauen, Deutschland.

出版信息

Hautarzt. 2018 Dec;69(12):1033-1038. doi: 10.1007/s00105-018-4267-6.

Abstract

Epidermodysplasia verruciformis (also known as Lewandowsky-Lutz dysplasia) is an extremely rare autosomal recessive genodermatosis, in which the skin is unusually sensitive to human papilloma viruses (HPV). It is associated with a high risk of developing non-melanocytic skin tumors. Treatment with keratolytic retinoids is currently considered to be the most effective therapy. Retinoids have a broad spectrum of activity and inhibit the growth of squamous cell carcinoma and other malignant tumors. We report the case of an 81-year-old woman who had been receiving prophylactic treatment with oral isotretinoin at a daily dose of 1.0-0.33 mg/kg bodyweight for about 18 years because of her epidermodysplasia verruciformis (HPV types 9 & 57 detected). We observed a reduction of the incidence of squamous cell carcinomas of the skin and presume a causal relationship between the treatment with this retinoid and the reduction of squamous cell carcinomas of the skin in this patient.

摘要

疣状表皮发育不良(也称为莱万多夫斯基-卢茨发育不良)是一种极其罕见的常染色体隐性遗传性皮肤病,其皮肤对人乳头瘤病毒(HPV)异常敏感。它与发生非黑素细胞性皮肤肿瘤的高风险相关。目前认为用角质溶解类视黄醇治疗是最有效的疗法。类视黄醇具有广泛的活性,可抑制鳞状细胞癌和其他恶性肿瘤的生长。我们报告了一例81岁女性病例,因其疣状表皮发育不良(检测到HPV 9型和57型),以每日1.0 - 0.33毫克/千克体重的剂量口服异维A酸进行预防性治疗约18年。我们观察到皮肤鳞状细胞癌的发病率有所降低,并推测该类视黄醇治疗与该患者皮肤鳞状细胞癌发病率降低之间存在因果关系。

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