Malignant Transformation in Porokeratosis Ptychotropica: A Systematic Review.

IF 3.5 4区 医学 Q1 DERMATOLOGY Acta dermato-venereologica Pub Date : 2024-10-10 DOI:10.2340/actadv.v104.40558
Chee H Loh, Chris L Tan, Kong B Tan, Holger Sudhoff, Peter Goon
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Abstract

Porokeratosis ptychotropica (PP) is a rare and unusual variant of porokeratosis. There is a dearth of information on the natural history, epidemiology, and optimal treatment options. This study aimed to characterize the worldwide distribution, epidemiology, clinical features, and treatments attempted for all reported cases of porokeratosis ptychotropica. A total of 59 cases of porokeratosis ptychotropica have been reported, with most cases originating from the United States. The median age of patients affected with porokeratosis ptychotropica was 49 years. The most involved body locations are the buttocks and gluteal cleft. The risk of malignant transformation in porokeratosis ptychotropica is approximately 1.7% but there is significant bias in estimating rare occurrences in rare diseases. In conclusion, PP is an important but under-recognized variant of porokeratosis, with a likely low risk of malignant transformation. The best available treatment modality remains uncertain; however, the use of topical lovastatin/cholesterol cream appears promising. Long-term surveillance appears prudent for porokeratosis ptychotropica due to a risk of cancerization.

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白斑角化病的恶性转化:系统综述。
脓疱型角化病(PP)是一种罕见的角化病变种。有关其自然史、流行病学和最佳治疗方案的信息十分匮乏。本研究旨在了解卟啉角化病在全球的分布、流行病学、临床特征以及所有已报道病例的治疗方法。据报道,目前共有59例角化病病例,其中大多数病例来自美国。porokeratosis ptychotropica患者的中位年龄为49岁。受累最多的身体部位是臀部和臀裂。角化病的恶性转化风险约为 1.7%,但在估计罕见疾病的罕见发生率时存在明显偏差。总之,PP 是一种重要的角化病变异,但未得到充分认识,恶性转化的风险可能很低。现有的最佳治疗方法仍不确定;不过,局部使用洛伐他汀/胆固醇霜似乎很有前景。由于脓疱型角化病有癌变的风险,因此对其进行长期监测显得非常谨慎。
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来源期刊
Acta dermato-venereologica
Acta dermato-venereologica 医学-皮肤病学
CiteScore
4.90
自引率
2.80%
发文量
210
审稿时长
6-12 weeks
期刊介绍: Acta Dermato-Venereologica publishes high-quality manuscripts in English in the field of Dermatology and Venereology, dealing with new observations on basic dermatological and venereological research, as well as clinical investigations. Each volume also features a number of Review articles in special areas, as well as short Letters to the Editor to stimulate debate and to disseminate important clinical observations. Acta Dermato-Venereologica has rapid publication times and is amply illustrated with a large number of colour photographs.
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