{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T08:07:59Z","timestamp":1753862879489,"version":"3.41.2"},"reference-count":33,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,9,18]],"date-time":"2024-09-18T00:00:00Z","timestamp":1726617600000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:sec><jats:title>Key Clinical Message<\/jats:title><jats:p>Diagnosis of Erdheim\u2013Chester disease (ECD) requires the clinician to be familiar with its various manifestations, classic radiologic and histologic features. This case highlights the significance of considering ECD in any patient presenting with bone pain and symmetric osteosclerosis of long bones of extremities to allow for early diagnosis and treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Abstract<\/jats:title><jats:p>Erdheim\u2013Chester disease (ECD) is a rare non\u2010Langerhans histiocytic disorder with diverse clinical manifestations, ranging from indolent, localized presentation to life\u2010threatening, multi\u2010systemic disease. Delayed or erroneous diagnosis is common. The presence of classic radiographic finding along with foamy histiocytes that is positive for CD68 but negative for CD1a on histologic examination establishes the diagnosis. We report a second case of ECD from Ethiopia. A 50\u2010year\u2010old Ethiopian man presented with a 13\u2010year history of bilateral lower leg bone pain, cold intolerance, somnolence, constipation, impotence, decreased libido, and secondary infertility. The diagnosis was suspected when skeletal X\u2010ray revealed bilateral symmetric sclerosis of metadiaphysis of femur, tibia, and humerus. The demonstration of foamy histiocytes that were positive for CD68 but negative for CD1a on histologic examination with immunohistochemical staining confirmed the diagnosis. Evaluation for the extent of the disease revealed coated aorta sign, hairy kidney sign, and cystic lesion with ground glass opacity of lung, primary hypothyroidism, and hypergonadotropic hypogonadism. ECD is rare histiocytic neoplasm with wide range of clinical features which often delay the diagnosis. Clinician should be mindful of the various presentations and the classic radiographic and histologic features of ECD. This case highlights the significance of entertaining ECD in any patient presenting with lower leg bone pain and symmetric osteosclerosis of long bones of lower extremities to allow for early diagnosis and treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ccr3.9447","type":"journal-article","created":{"date-parts":[[2024,9,19]],"date-time":"2024-09-19T06:39:05Z","timestamp":1726727945000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Erdheim\u2013Chester disease: An elusive diagnosis in a 50\u2010year\u2010old Ethiopian man presenting with diffuse sclerotic bone lesion"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4221-5694","authenticated-orcid":false,"given":"Semir Abdi","family":"Usmael","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Addisu Alemu","family":"Gebrehiywot","sequence":"additional","affiliation":[{"name":"Department of Pathology Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashenafi Lemma","family":"Bekele","sequence":"additional","affiliation":[{"name":"Department of Radiology Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3298-1491","authenticated-orcid":false,"given":"Solomon Bishaw","family":"Yezengaw","sequence":"additional","affiliation":[{"name":"Department of Radiology Jugel General Hospital  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tekalign Tsegaye","family":"Tefera","sequence":"additional","affiliation":[{"name":"Department of Orthopedic and Trauma Surgery Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hunduma Bikila","family":"Bote","sequence":"additional","affiliation":[{"name":"Department of Orthopedic and Trauma Surgery Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kalkidan Abera","family":"Shibeshi","sequence":"additional","affiliation":[{"name":"Department of Radiology Harar General Hospital  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anteneh Belachew","family":"Fantaye","sequence":"additional","affiliation":[{"name":"Department of Pathology Haramaya University College of Health and Medical Science  Harar Ethiopia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,9,18]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_13_2_1","DOI":"10.1007\/BF01942684"},{"unstructured":"World Health Organization.WHO classification of tumours of haematopoietic and lymphoid tissues. 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