{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T11:20:33Z","timestamp":1767093633072,"version":"3.44.0"},"reference-count":10,"publisher":"BMJ","issue":"8","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2023,7,25]]},"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:p>Langerhans cell histiocytosis is a great imitator of other diseases with an often-delayed diagnosis leading to a concerning delay in commencing treatment. We present the case of a male who was previously diagnosed with Hailey-Hailey disease, in whom several treatment options had failed, was referred to the dermatology team for evaluation of a 3-month atypical, extensive, painful and pruritic cutaneous flexural eruption. On systems review, he reported a 2-year history of polyuria and polydipsia. Repeat skin biopsy revealed a prominent histiocytic infiltrate on histopathology with corresponding positive expression of Langerin (CD207), S100, CyclinD1 and p-ERK on immunohistochemistry staining. An MRI of the brain demonstrated posterior pituitary enhancement. The clinical presentation, biopsy and investigations confirmed a diagnosis of a multisystem Langerhans cell histiocytosis, which resulted in longstanding patient morbidity. With considerable multidisciplinary teamwork, a gradual and sustained resolution of his lesions, pain, polyuria and polydipsia was achieved.<\/jats:p>","DOI":"10.1136\/bcr-2022-254205","type":"journal-article","created":{"date-parts":[[2023,8,4]],"date-time":"2023-08-04T11:45:15Z","timestamp":1691149515000},"page":"e254205","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Adult multisystem Langerhans cell histiocytosis with cutaneous involvement manifesting as extensive flexural erosions"],"prefix":"10.1136","volume":"16","author":[{"given":"Lachlan Dat Wah","family":"Lau","sequence":"first","affiliation":[{"name":"Department of Dermatology, Western Health, Footscray, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mi","family":"Vu","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Western Health, Footscray, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alex","family":"Nirenberg","sequence":"additional","affiliation":[{"name":"Department of Pathology, Western Health, Footscray, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Scardamaglia","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Western Health, Footscray, Victoria, Australia"},{"name":"Department of Medicine, Faculty of Medicine Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2023,8,4]]},"reference":[{"key":"2025082722254406000_16.8.e254205.1","doi-asserted-by":"crossref","unstructured":"Langerhans cell Histiocytosis in adults: Histiocytosis Association. 2022. Available: https:\/\/histio.org\/langerhans-cell-histiocytosis-in-adults\/","DOI":"10.53347\/rID-151596"},{"key":"2025082722254406000_16.8.e254205.2","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1007\/s40257-019-00477-z","article-title":"Hailey\u2013hailey disease: an update review with a focus on treatment data","volume":"21","author":"Ben Lagha","year":"2020","journal-title":"Am J Clin Dermatol"},{"key":"2025082722254406000_16.8.e254205.3","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1016\/j.hoc.2015.06.005","article-title":"Clinical characteristics and treatment of Langerhans cell histiocytosis","volume":"29","author":"Monsereenusorn","year":"2015","journal-title":"Hematol Oncol Clin North Am"},{"key":"2025082722254406000_16.8.e254205.4","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000013366","article-title":"Adult Langerhans' cell histiocytosis with multisystem involvement: a case report","volume":"97","author":"Kim","year":"2018","journal-title":"Medicine (Baltimore)"},{"key":"2025082722254406000_16.8.e254205.5","first-page":"104","article-title":"Primary oro-facial manifestations of Langerhans cell histiocytosis in pediatric age: a bi-institutional retrospective study on 45 cases","volume":"7","author":"Capodiferro","year":"2020","journal-title":"Children (Basel)"},{"key":"2025082722254406000_16.8.e254205.6","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-8-72"},{"key":"2025082722254406000_16.8.e254205.7","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1002\/pbc.25834","article-title":"Diverse cutaneous presentations of Langerhans cell histiocytosis in children: a retrospective cohort study","volume":"63","author":"Morren","year":"2016","journal-title":"Pediatr Blood Cancer"},{"key":"2025082722254406000_16.8.e254205.8","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2015.65.9508"},{"key":"2025082722254406000_16.8.e254205.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2015.12.029"},{"key":"2025082722254406000_16.8.e254205.10","doi-asserted-by":"crossref","first-page":"2601","DOI":"10.1182\/blood.2021014343","article-title":"International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults","volume":"139","author":"Goyal","year":"2022","journal-title":"Blood"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2022-254205","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T05:25:55Z","timestamp":1756358755000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2022-254205"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,8]]},"references-count":10,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2023,8,4]]},"published-print":{"date-parts":[[2023,8]]}},"alternative-id":["10.1136\/bcr-2022-254205"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2022-254205","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2023,8]]}}}