Chee H Loh, Chris L Tan, Kong B Tan, Holger Sudhoff, Peter Goon
{"title":"Malignant Transformation in Porokeratosis Ptychotropica: A Systematic Review.","authors":"Chee H Loh, Chris L Tan, Kong B Tan, Holger Sudhoff, Peter Goon","doi":"10.2340/actadv.v104.40558","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":6944,"journal":{"name":"Acta dermato-venereologica","volume":null,"pages":null},"PeriodicalIF":3.5000,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11481308/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta dermato-venereologica","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.2340/actadv.v104.40558","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"DERMATOLOGY","Score":null,"Total":0}
引用次数: 0
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.
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.