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The objective of this study is to elucidate the dermatologic manifestations, associated symptoms, pathophysiology and management recommendations of HH. We searched five primary databases (PubMed, Embase, Cochrane Library, Scopus and Web of Science) up to April 2023. Non\u2010English articles were included to minimize language bias. The studies were evaluated using Oxford Centre for Evidence\u2010based Medicine standards, with adherence to PRISMA guidelines. Inaccessible articles were directly sourced from authors. Out of the initial 1582 publications from 1904 to 2023, 22 studies (19 in English, 2 in French and 1 in German) were selected. Most reports were from the USA, UK and France and were predominantly case reports, covering 148 patients with skin symptoms related to hereditary hemochromatosis. We collected data on the cutaneous findings and, when available, their histopathological features. The current study highlights the scope, variety and traits of dermatologic symptoms in hereditary hemochromatosis, pinpointing research gaps and areas for future exploration. Our review accentuates the diverse dermatological manifestations of hereditary hemochromatosis, notably hyperpigmentation, hypertrichosis and resistant pruritus, often linked to excessive iron deposition and subsequent impairment of skin cell function. We also found controversial evidence indicating that skin cancers seem to be associated with hereditary hemochromatosis. Porphyria cutanea tarda and hereditary hemochromatosis were frequently reported together. Given hereditary hemochromatosis's genetic nature, early identification in one individual can substantially guide familial care and preemptive interventions. Clinicians should prioritize hereditary hemochromatosis as a differential when patients present with specific dermatological symptoms, especially in sun\u2010exposed regions. A rigorous assessment ensures accurate diagnosis, facilitating optimal management for both the patient and their family.<\/jats:p>","DOI":"10.1111\/jdv.20098","type":"journal-article","created":{"date-parts":[[2024,5,16]],"date-time":"2024-05-16T11:45:15Z","timestamp":1715859915000},"page":"976-986","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Dermatologic manifestations of hereditary hemochromatosis: A systematic review"],"prefix":"10.1111","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2018-6378","authenticated-orcid":false,"given":"Hossein","family":"Akbarialiabad","sequence":"first","affiliation":[{"name":"Faculty of Medicine, UNSW Medicine University of New South Wales  Sydney New South Wales Australia"},{"name":"Department of Dermatology St George Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2295-5081","authenticated-orcid":false,"given":"Parnian","family":"Jamshidi","sequence":"additional","affiliation":[{"name":"School of Medicine Shahid Beheshti University of Medical Sciences  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-8478-4161","authenticated-orcid":false,"given":"Jeffrey P.","family":"Callen","sequence":"additional","affiliation":[{"name":"Division of Dermatology, Department of Medicine University of Louisville  Louisville Kentucky USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2971-0199","authenticated-orcid":false,"given":"D\u00e9d\u00e9e F.","family":"Murrell","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, UNSW Medicine University of New South Wales  Sydney New South Wales Australia"},{"name":"Department of Dermatology St George Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,5,16]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-7471-9_19"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa041534"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.280.2.172"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.12347"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1006\/bcmd.2002.0585"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0896-399"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-019-1097-2"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.24330"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-129-11_Part_2-199812011-00003"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1977.01640020033004"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/bjd\/ljad113.199"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.2910600204"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.07.029"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.publhealth.21.1.65"},{"issue":"10","key":"e_1_2_12_16_1","first-page":"44","article-title":"Acquired hemochromatosis with pronounced pigment deposition of the upper eyelids","volume":"6","author":"Chacon AH","year":"2013","journal-title":"J Clin Aesthet Dermatol"},{"issue":"3","key":"e_1_2_12_17_1","first-page":"183","article-title":"Hereditary hemochromatosis","volume":"87","author":"Crownover BK","year":"2013","journal-title":"Am Fam Physician"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1159\/000243802"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13643-021-01626-4"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-151-4-200908180-00135"},{"key":"e_1_2_12_21_1","unstructured":"Oxford Centre for Evidence\u2010Based Medicine.Oxford Centre for Evidence\u2010Based Medicine: Levels of evidence (March 2009). 2009 [cited 2023 Aug 12]. 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