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Here, we report a case of polydactylous SCCNU and perform a comprehensive review of MEDLINE and Embase to collate 44 cases of polydactylous SCCNU reported to date. Polydactylous patients were younger on average (48 to 61-63 years) and had a longer diagnostic delay (44 vs 35.1 months) compared with reported monodactylous cases. Human papillomavirus (HPV) positivity was observed in 49% of cases, and the most common serotypes noted were 16 (25.8%), 73 (16.1%), 58 (9.7%), 18 (6.5%), and 33 (6.5%). Twenty percent of the cases were in immunosuppressed individuals who had a statistically significant lower age at diagnosis (39.33 years vs 51.12 years; P = .01) and diagnostic delay (2.50 months vs 132.46 months, P = .04). Patients with HPV positivity had a lower age at diagnosis (43.74 years vs 53.29 years, P = .04). Environmental exposures noted to be associated with polydactylous disease included X-rays, paint\/solvents, soluble oils, and stagnant water. This comprehensive literature review serves to characterize polydactylous SCCNU and distinguish the differences in its characteristics to improve diagnosis and clinical recognition.<\/jats:p>","DOI":"10.1177\/1203475420977474","type":"journal-article","created":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T02:43:41Z","timestamp":1606790621000},"page":"303-314","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":7,"title":["Polydactylous Squamous Cell Carcinoma of the Nail Unit: A Structured Review of the Literature"],"prefix":"10.1177","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8950-0965","authenticated-orcid":false,"given":"Patrick M.","family":"Jedlowski","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Dermatology, University of Arizona College of Medicine, Tucson, AZ, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahdieh F.","family":"Jedlowski","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Dermatology, University of Arizona College of Medicine, Tucson, AZ, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Segal","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Dermatology, University of Arizona College of Medicine, Tucson, AZ, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2020,11,30]]},"reference":[{"key":"bibr1-1203475420977474","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhsa.2018.01.010"},{"key":"bibr2-1203475420977474","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1970.04000010065011"},{"issue":"1","key":"bibr3-1203475420977474","first-page":"108","volume":"30","author":"Siegel H","year":"1937","journal-title":"Cancer Research"},{"key":"bibr4-1203475420977474","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2013.02.008"},{"key":"bibr5-1203475420977474","doi-asserted-by":"publisher","DOI":"10.5826\/dpc.0803a017"},{"key":"bibr6-1203475420977474","first-page":"e9","volume":"11","author":"Abner S.","year":"2011","journal-title":"Eplasty"},{"key":"bibr7-1203475420977474","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2003.184"},{"key":"bibr8-1203475420977474","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(87)70191-1"},{"key":"bibr9-1203475420977474","first-page":"229","volume":"3","author":"A. 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