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Extragenital LS (EGLS) can occur simultaneously with genital form; however, in 6% of the cases, only extragenital form has been described. Genetic, autoimmune, infectious, environmental and hormonal factors are implicated in its aetiology. Extragenital LS presents as asymptomatic white opalescent papules, which cluster in plaques and slowly progress over time resulting in parchment\u2010like skin usually involving upper trunk, neck and shoulders. Lesions are frequently accompanied by purpura\/haemorrhagic spots. The relationship with morphoea has been a topic of debate. Association with several autoimmune diseases has been observed. Diagnosis is usually based on clinical and dermoscopic examination and further supported by histopathological findings. LS needs to be differentiated from several other dermatological conditions such as discoid lupus erythematosus, vitiligo, mycosis fungoides (hypopigmented variant), lichen planus, graft\u2010versus\u2010host disease and morphoea depending upon the stage of the disease. Generally, extragenital LS is believed to lack carcinogenic potential. However, case reports with possible malignant transformation have been described. In this article, the authors have described a concise review of the extragenital form of LS.<\/jats:p>","DOI":"10.1111\/ajd.13890","type":"journal-article","created":{"date-parts":[[2022,8,11]],"date-time":"2022-08-11T13:30:02Z","timestamp":1660224602000},"page":"452-462","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":56,"title":["Extragenital lichen sclerosus: A comprehensive review"],"prefix":"10.1111","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7965-5194","authenticated-orcid":false,"given":"Tasleem","family":"Arif","sequence":"first","affiliation":[{"name":"Ellahi Medicare Clinic  Srinagar Kashmir India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafiya","family":"Fatima","sequence":"additional","affiliation":[{"name":"Department of Dermatology Tadawi General Hospital  Dammam Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marwa","family":"Sami","sequence":"additional","affiliation":[{"name":"Ellahi Medicare Clinic  Srinagar Kashmir India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,8,11]]},"reference":[{"key":"e_1_2_19_2_1","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.174096"},{"key":"e_1_2_19_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0190-9622(95)90060-8"},{"key":"e_1_2_19_4_1","first-page":"126","article-title":"Lichen sclerosus","volume":"108","author":"Jab\u0142onowska O","year":"2021","journal-title":"Dermatol Rev\/Przegl Dermatol"},{"key":"e_1_2_19_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)08228-2"},{"key":"e_1_2_19_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40257-012-0006-4"},{"key":"e_1_2_19_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.juro.2007.08.024"},{"key":"e_1_2_19_8_1","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.160516"},{"key":"e_1_2_19_9_1","first-page":"240","article-title":"Ichthyosis of the tongue and vulva","volume":"246","author":"Weir RF","year":"1875","journal-title":"NY State J Med"},{"key":"e_1_2_19_10_1","first-page":"447","article-title":"Lichen plan sclereux","volume":"20","author":"Hallopeau H","year":"1889","journal-title":"Ann Derm Syph (2nd series)"},{"key":"e_1_2_19_11_1","first-page":"57","article-title":"Sur un nouveau cas de lichen plan atrophique","volume":"27","author":"Hallopeau H","year":"1896","journal-title":"Ann Derm Syph (3rd series)"},{"key":"e_1_2_19_12_1","first-page":"358","article-title":"Sur un nouveau cas de lichen de Wilson sclereux","volume":"9","author":"Hallopeau H","year":"1898","journal-title":"Ann Derm Syph (3rd series)"},{"key":"e_1_2_19_13_1","first-page":"472.154","article-title":"Lec;ons cliniques sur les maladees cutantes et syphiliques","volume":"43","author":"Darier J","year":"1887","journal-title":"Union Med Can"},{"key":"e_1_2_19_14_1","first-page":"147","article-title":"Lichen sclerosus","volume":"17","author":"Friedrich EG","year":"1976","journal-title":"J Reprod Med"},{"key":"e_1_2_19_15_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2230.2003.01211.x"},{"key":"e_1_2_19_16_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2000.03360.x"},{"key":"e_1_2_19_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140\u20106736(03)13863\u20109"},{"issue":"2","key":"e_1_2_19_18_1","first-page":"111","article-title":"Concomitant morphea and lichen sclerosus et atrophicus in the same plaque at the site of intramuscular drug injection: an interesting case presentation","volume":"27","author":"Arif T","year":"2018","journal-title":"Acta Dermatovenerol Alp Pannonica Adriat"},{"key":"e_1_2_19_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.1994.tb00386.x"},{"key":"e_1_2_19_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000372-198710000-00002"},{"key":"e_1_2_19_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000372-199008000-00005"},{"issue":"319","key":"e_1_2_19_22_1","first-page":"22","article-title":"Epstein\u2010Barr virus and human papillomavirus infection in vulvar lichen sclerosus","volume":"14","author":"Aide\u00b4 S","year":"2010","journal-title":"J Low Genit Tract Dis"},{"key":"e_1_2_19_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102230.2004.01460"},{"key":"e_1_2_19_24_1","first-page":"262","article-title":"Lichen sclerosus and the K\u00f6bner phenomenon","volume":"19","author":"Todd P","year":"1994","journal-title":"ClinExp Dermatol"},{"key":"e_1_2_19_25_1","first-page":"573527","article-title":"Development of extragenital lichen Sclerosus in malignant melanoma patients treated with ipilimumab in combination with nivolumab. 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