{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T00:06:04Z","timestamp":1756339564635,"version":"3.44.0"},"reference-count":20,"publisher":"BMJ","issue":"3","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,2,13]]},"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:p>With fewer than 40 documented cases, temporal bone Langerhans cell histiocytosis (LCH) is exceptionally rare in adults. We present a case of temporal bone and uterine cervix LCH in an adult with concurrent cutaneous Erdheim-Chester disease. A woman in her 50s presented with several months of unilateral right aural fullness, otalgia, otorrhoea and tinnitus, unresponsive to antibiotics and aural irrigation. CT of the temporal bone revealed right mastoid destruction, prompting further investigation. Histology obtained from mastoid exploration confirmed LCH. Initial management included radiotherapy, followed by methotrexate and later, cytarabine. Disease monitoring was conducted withPositron Emision Tomography-CT. Due to its rarity, LCH is often initially misdiagnosed. Clinicians should remain vigilant for LCH in patients presenting with unresolved otological symptoms that deviate from the expected clinical course. Swift communication between relevant medical teams is essential for rapid diagnosis and optimal management of potential multisystem LCH when symptoms present in different areas.<\/jats:p>","DOI":"10.1136\/bcr-2024-263493","type":"journal-article","created":{"date-parts":[[2025,3,28]],"date-time":"2025-03-28T03:41:39Z","timestamp":1743133299000},"page":"e263493","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Temporal bone Langerhans cell histiocytosis with uterine cervix involvement and cutaneous Erdheim-Chester disease"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-5985-6003","authenticated-orcid":false,"given":"Sara","family":"Lim","sequence":"first","affiliation":[{"name":"Otorhinolaryngology, NHS Greater Glasgow and Clyde, Glasgow, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niall","family":"Woodley","sequence":"additional","affiliation":[{"name":"Otorhinolaryngology, NHS Greater Glasgow and Clyde, Glasgow, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ida","family":"Amir","sequence":"additional","affiliation":[{"name":"King\u2019s College Hospital London, Dubai, UAE"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,3,27]]},"reference":[{"key":"2025082710393908000_18.3.e263493.1","unstructured":"Kumar V , Abbas AK , Aster JC . Robbins & Cotran pathologic basis of disease. 10th edn. Philadelphia, PA: Elsevier, 2021:627."},{"key":"2025082710393908000_18.3.e263493.2","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1016\/j.radcr.2019.03.040","article-title":"Langerhans\u2019 cell histiocytosis of the temporal bone in an adult with central diabetes insipidus","volume":"14","author":"Allen","year":"2019","journal-title":"Radiol Case Rep"},{"key":"2025082710393908000_18.3.e263493.3","doi-asserted-by":"publisher","DOI":"10.2174\/1874440001307010053"},{"key":"2025082710393908000_18.3.e263493.4","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1177\/01945998211004590","article-title":"Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review","volume":"166","author":"Chen","year":"2022","journal-title":"Otolaryngol Head Neck Surg"},{"key":"2025082710393908000_18.3.e263493.5","doi-asserted-by":"publisher","DOI":"10.31486\/toj.19.0032"},{"key":"2025082710393908000_18.3.e263493.6","article-title":"Otic langerhans\u2019 cell histiocytosis in an adult: a case report and review of the literature","volume":"2013","author":"Gungadeen","year":"2013","journal-title":"Case Rep Otolaryngol"},{"key":"2025082710393908000_18.3.e263493.7","first-page":"294","article-title":"Systemic Langerhans Cell Histiocytosis with Bilateral Temporal Bone Involvement in an Adult","volume":"9","author":"Stiller-Timor","year":"2013","journal-title":"Int Adv Otol"},{"key":"2025082710393908000_18.3.e263493.8","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbo.2022.100454","article-title":"Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns","volume":"36","author":"Hu","year":"2022","journal-title":"J Bone Oncol"},{"key":"2025082710393908000_18.3.e263493.9","doi-asserted-by":"crossref","DOI":"10.1007\/s11912-019-0810-6","article-title":"Systemic Histiocytosis (Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Destombes-Rosai-Dorfman Disease): from Oncogenic Mutations to Inflammatory Disorders","volume":"21","author":"Papo","year":"2019","journal-title":"Curr Oncol Rep"},{"key":"2025082710393908000_18.3.e263493.10","article-title":"A Rare Case of Erdheim-Chester Disease (Non-Langerhans Cell Histiocytosis) with Concurrent Langerhans Cell Histiocytosis: A Diagnostic and Therapeutic Challenge","volume":"2018","author":"Hashmi","year":"2018","journal-title":"Case Rep Hematol"},{"key":"2025082710393908000_18.3.e263493.11","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.gore.2017.08.005","article-title":"Langerhans cell histiocytosis limited to the female genital tract: A review of literature with three additional cases","volume":"22","author":"Wieland","year":"2017","journal-title":"Gynecol Oncol Rep"},{"key":"2025082710393908000_18.3.e263493.12","doi-asserted-by":"publisher","DOI":"10.1136\/ijgc-00009577-200305000-00021"},{"key":"2025082710393908000_18.3.e263493.13","doi-asserted-by":"publisher","DOI":"10.1002\/lary.25773"},{"key":"2025082710393908000_18.3.e263493.14","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"},{"key":"2025082710393908000_18.3.e263493.15","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1002\/mpo.2950250204","article-title":"Oral methotrexate and alternate-day prednisone for low-risk Langerhans cell histiocytosis","volume":"25","author":"Womer","year":"1995","journal-title":"Med Pediatr Oncol"},{"key":"2025082710393908000_18.3.e263493.16","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.2001.04298.x"},{"key":"2025082710393908000_18.3.e263493.17","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1001\/jamaoncol.2017.5029","article-title":"Vemurafenib for BRAF V600-Mutant Erdheim-Chester Disease and Langerhans Cell Histiocytosis: Analysis of Data From the Histology-Independent, Phase 2, Open-label VE-BASKET Study","volume":"4","author":"Diamond","year":"2018","journal-title":"JAMA Oncol"},{"key":"2025082710393908000_18.3.e263493.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijporl.2009.07.010"},{"key":"2025082710393908000_18.3.e263493.19","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1179\/1754762812Y.0000000023","article-title":"Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis","volume":"14","author":"Segel","year":"2013","journal-title":"Cochlear Implants Int"},{"key":"2025082710393908000_18.3.e263493.20","doi-asserted-by":"crossref","first-page":"70","DOI":"10.5152\/iao.2023.22716","article-title":"Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis","volume":"19","author":"Gendre","year":"2023","journal-title":"J Int Adv Otol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2024-263493","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T17:39:50Z","timestamp":1756316390000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2024-263493"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3]]},"references-count":20,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2025,3,27]]},"published-print":{"date-parts":[[2025,3]]}},"alternative-id":["10.1136\/bcr-2024-263493"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2024-263493","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2025,3]]}}}