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The earliest descriptions of this condition were provided in the early 20th century, yet it remains relatively unknown, with approximately 200 cases since described. Most commonly seen in older women, <jats:styled-content style=\"fixed-case\">TTS<\/jats:styled-content> characteristically involves persistent facial ulceration with loss of sensation and paresthesia along the distribution of the trigeminal dermatome. Ulceration often occurs in the alar region, following self\u2010manipulation in response to paresthesias. Time of onset of <jats:styled-content style=\"fixed-case\">TTS<\/jats:styled-content> after trigeminal insult may vary from weeks to decades, and emergence of ulceration may be associated with psychiatric disorders. Diagnosis is clinical and made by exclusion of similarly presenting conditions. Histology is nonspecific yet necessary to exclude other causes of facial ulceration. Although there is not yet a standard management strategy, a number of successful approaches have been reported including pharmaceutical and surgical interventions, installation of a protector, and transcutaneous nerve stimulation. However, because of the self\u2010inflicted manifestations of this disorder, behavioral modifications remain of the utmost importance. This review serves to address the history, epidemiology, pathogenesis, clinical presentation, histology, diagnosis, differential diagnosis, and management options for <jats:styled-content style=\"fixed-case\">TTS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/ijd.14098","type":"journal-article","created":{"date-parts":[[2018,6,22]],"date-time":"2018-06-22T04:35:00Z","timestamp":1529642100000},"page":"530-537","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":57,"title":["Trigeminal trophic syndrome: an updated review"],"prefix":"10.1111","volume":"58","author":[{"given":"Ayesha U.","family":"Khan","sequence":"first","affiliation":[{"name":"University of Cincinnati College of Medicine Cincinnati OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amor","family":"Khachemoune","sequence":"additional","affiliation":[{"name":"Veterans Affairs Medical Center Brooklyn NY USA"},{"name":"Department of Dermatology SUNY Downstate Brooklyn NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,6,21]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-1318"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1524-4725.2004.30220.x"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/1346-8138.12490"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.4065\/83.4.502"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-3083.2007.02250.x"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.2165\/11594060-000000000-00000"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-2982.2008.01636.x"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4632.2006.02943.x"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0306-4522(99)00115-3"},{"key":"e_1_2_13_11_1","doi-asserted-by":"crossref","first-page":"26","DOI":"10.5070\/D319D519F7","article-title":"Trigeminal trophic syndrome: a report of two cases with a review of literature","volume":"9","author":"Elloumi\u2010Jellouli A","year":"2003","journal-title":"Dermatol Online J"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1532-5415.2005.53358.x"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.3171\/JNS\/2008\/108\/01\/0170"},{"key":"e_1_2_13_14_1","first-page":"292","article-title":"Trigeminal trophic syndrome associated with the use of synthetic marijuana","volume":"17","author":"Khan FA","year":"2017","journal-title":"Ochsner J"},{"key":"e_1_2_13_15_1","first-page":"291","article-title":"Trigeminal trophic syndrome: report of 3 cases affecting the scalp","volume":"92","author":"Bolaji RS","year":"2013","journal-title":"Cutis"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-2675"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/lary.21421"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0278-2391(96)90758-2"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.1979.04010090060029"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/IOP.0000000000000766"},{"key":"e_1_2_13_21_1","doi-asserted-by":"crossref","first-page":"12","DOI":"10.5070\/D3218028437","article-title":"A rare case of trigeminal trophic syndrome with an extensive scalp, forehead, and upper eyelid ulceration in a patient with undiagnosed Alzheimer disease","volume":"21","author":"Incel Uysal P","year":"2015","journal-title":"Dermatol Online J"},{"key":"e_1_2_13_22_1","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1001\/archderm.1996.03890300021002","article-title":"Herpetic trigeminal trophic syndrome. 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