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A systematic review following PRISMA guidelines was performed of articles published prior to March 2021. Articles were included in the review if a full\u2010text English version was available. Of the surveyed literature, 27 full\u2010text case reports were included in the final analysis. Benign eccrine poroma and porocarcinoma were the most common tumor subtypes (nine and eight cases, respectively). Males were only affected by poroma and porocarcinoma, while females were affected by all tumor subtypes. The first toe was the most common lower extremity affected. Misdiagnosis led to delayed treatment in 25% of cases. As such, while periungual eccrine neoplasms are rare diagnoses, the nonspecific presentations of these growths raise concerns about misdiagnosis and delayed treatment. Further research is needed related to sex\u2010differences in the epidemiology of these growths and into the prevalence of the first toe as a location. These tumors should be considered in the differential diagnosis for nail unit afflictions.<\/jats:p>","DOI":"10.1111\/ijd.15893","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T08:24:06Z","timestamp":1631089446000},"page":"812-820","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A systematic review of periungual eccrine neoplasms"],"prefix":"10.1111","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6455-3243","authenticated-orcid":false,"given":"Elizabeth M.","family":"Rao","sequence":"first","affiliation":[{"name":"Department of Dermatology MetroHealth System  Cleveland OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas J.","family":"Knackstedt","sequence":"additional","affiliation":[{"name":"Department of Dermatology MetroHealth System  Cleveland OH USA"},{"name":"School of Medicine Case Western Reserve University  Cleveland OH 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