Department of Dermatology, National University Hospital, Singapore.
Department of Pathology, National University Hospital, Singapore.
Acta Derm Venereol. 2024 Oct 10;104:adv40558. doi: 10.2340/actadv.v104.40558.
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.
播散性浅表性光线性汗孔角化症（PP）是一种罕见且不常见的汗孔角化症变异型。关于其自然病史、流行病学和最佳治疗选择的信息很少。本研究旨在描述所有报道的播散性浅表性光线性汗孔角化症病例的全球分布、流行病学、临床特征和尝试的治疗方法。共报道了 59 例播散性浅表性光线性汗孔角化症，大多数病例来自美国。播散性浅表性光线性汗孔角化症患者的中位年龄为 49 岁。最常见的受累部位是臀部和臀沟。播散性浅表性光线性汗孔角化症恶变的风险约为 1.7%，但在罕见疾病中估计罕见事件存在很大的偏差。总之，PP 是一种重要但认识不足的汗孔角化症变异型，恶变风险可能较低。最佳的治疗方法仍不确定；然而，局部使用洛伐他汀/胆固醇乳膏似乎很有前途。由于有癌变的风险，对播散性浅表性光线性汗孔角化症进行长期监测似乎是谨慎的。
