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This systematic review summarizes 59 reported cases worldwide, most originating from the United States, with a median patient age of 49 years and frequent involvement of the buttocks and gluteal cleft. Malignant transformation risk is estimated at ~1.7%, with major uncertainty due to rarity and study bias. Topical lovastatin/cholesterol cream appears promising, and long-term surveillance is recommended to reduce delayed cancerization.",{"@graph":69,"@context":126},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/malignant-transformation-in-porokeratosis-ptychotropica-a-systematic-review/329151/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/malignant-transformation-in-porokeratosis-ptychotropica-a-systematic-review/329151.png","ImageObject",300,407,{"name":92,"@type":93},"River Wang","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-24","2026-09-21",true,{"@type":102,"interactionType":103,"userInteractionCount":29},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118,122],{"name":109,"@type":110,"acceptedAnswer":111},"What is porokeratosis ptychotropica (PP)?","Question",{"text":112,"@type":113},"PP is a rare and unusual variant of porokeratosis that commonly involves the genitals and gluteal folds, including buttocks and the perianal area.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How many cases were identified and where do they mostly come from?",{"text":117,"@type":113},"The review identified 59 reported cases worldwide, with most originating from the United States.",{"name":119,"@type":110,"acceptedAnswer":120},"What is the estimated risk of malignant transformation in PP?",{"text":121,"@type":113},"The risk is approximately 1.7%, though the estimate has significant bias because of the challenges in studying extremely rare diseases.",{"name":123,"@type":110,"acceptedAnswer":124},"Which treatment appears promising, and why is follow-up important?",{"text":125,"@type":113},"Topical lovastatin/cholesterol cream appears promising, but the optimal treatment remains uncertain. Long-term surveillance is considered prudent due to the risk of cancerization.","https://schema.org",{"og:url":83,"og:type":128,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":130,"canonical":83},"index,follow",{"doc_id":132,"site_id":62},329151,1790240968,{"code":4,"msg":5,"data":135},{"doc_id":132,"user_id":136,"nickname":92,"user_avatar":137,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":138,"file_id":139,"file_url":140,"file_type":141,"file_size":142,"view_count":29,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":29,"language":143,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":144,"faqs":145,"seo_title":146,"seo_description":67,"update_tm":147,"read_time":148},1099514067438,"https://ap-avatar.wpscdn.com/avatar/100002539ee87300030?x-image-process=image/resize,m_fixed,w_180,h_180&k=1780474512215547542","1/6  \nREVIEW ARTICLE  \nMalignant Transformation in Porokeratosis Ptychotropica: A Systematic Review  \nChee H. LOH1, Chris L. TAN1, Kong B. TAN2, Holger SUDHOFF3 and Peter GOON1,4  \n1Department of Dermatology, National University Hospital, Singapore, 2Department of Pathology, National University Hospital, Singapore, 3Department of Otolaryngology, University of Bielefeld, Germany, and 4Department of Medicine, National University of Singapore, Singapore  \nPorokeratosis 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.  \nKey words: porokeratosis; porokeratosis ptychotropica; genitogluteal porokeratosis  \nSubmitted Apr 17, 2024. Accepted after revision Aug 6, 2024 Published Oct 10, 2024. DOI: 10.2340/actadv.v104.40558 Acta Derm Venereol 2024; 104: adv40558 .  \nCorr: Chee H. Loh, Department of Dermatology, NUHS Tower Block Level 10, 1E Kent Ridge Road, Singapore 119228. E-mail: cheehoou.loh@ [mohh.com.sg](mohh.com.sg)  \nPorokerdistincattpseisnosyapegsesnuodermch as diastsoesinwathd sculipnecfiaclilyal actinic porokeratosis (DSAP), disseminated superficial porokeratosis (DSP), linear porokeratosis (LP), punctate porokeratosis (PuP), porokeratosis of Mibelli (PM), and porokeratosis ptychotropica (PP). PPis a rare and unusual variant of porokeratosis that involves the genitals and gluteal folds, buttocks, and perianal area.  \nThe PP variant is the most infrequently reported of all the phenotypes, as it involves the perineal and perianal areas, and therefore has a resultant concomitant lack of ultraviolet (UV) exposure in most people. UV exposure stands out as the most important mutagen for skin malignancies. Indeed, a recent study (1) concluded that patients with porokeratoses had a much-increased risk  \nSIGNIFICANCE  \nPorokeratosis ptychotropica is a rare disease affecting the genitogluteal region. The natural history, epidemiology, and optimal treatment options are not well understood. We confirmed that the reported worldwide frequency of porokeratosis ptychotropica is extremely low. Porokeratosis ptychotropica also appears to be associated with a lower risk of malignant transformation compared with other commoner forms of the disease but there are inherent difficulties in studying the natural history of extremely rare diseases. The most involved body locations were the buttocks and gluteal cleft. The optimal treatment option remains uncertain, but topical lovastatin/cholesterol cream is a promising treatment option. It is important to be cognizant of this under-recognized condition to avoid delayed diagnosis.  \nof developing skin malignancies (hazard ratio of 4.3 for squamous cell carcinoma, 2.4 for basal cell carcinoma, and 1.83 for melanoma) . Inci et al. (1) reported only 2 cases of the genitogluteal or PP variant out of a cohort of 108 (1.8%) cases collected from 2016 to 2020.  \nThe term “porokeratosis ptychotropica” is derived from the Greek w","cbCaii1owQcnod84","https://ap.wps.com/l/cbCaii1owQcnod84","pdf",1068570,"English","# Porokeratosis ptychotropica and study aims\n# Worldwide case distribution and epidemiology\n# Clinical features and affected body locations\n# Malignant transformation risk and significance\n# Treatment options and evidence for topical lovastatin/cholesterol cream\n# Case history and clinical presentation","[{\"question\":\"What is porokeratosis ptychotropica (PP)?\",\"answer\":\"PP is a rare and unusual variant of porokeratosis that commonly involves the genitals and gluteal folds, including buttocks and the perianal area.\"},{\"question\":\"How many cases were identified and where do they mostly come from?\",\"answer\":\"The review identified 59 reported cases worldwide, with most originating from the United States.\"},{\"question\":\"What is the estimated risk of malignant transformation in PP?\",\"answer\":\"The risk is approximately 1.7%, though the estimate has significant bias because of the challenges in studying extremely rare diseases.\"},{\"question\":\"Which treatment appears promising, and why is follow-up important?\",\"answer\":\"Topical lovastatin/cholesterol cream appears promising, but the optimal treatment remains uncertain. Long-term surveillance is considered prudent due to the risk of cancerization.\"}]","Malignant Transformation in Porokeratosis Ptychotropica - A Systematic Review | PDF",1789982763,15]