Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia
Erdheim-Chester disease (ECD) or lipoid granulomatosis is a rare non-langerhan cell histiocytosis disease characterised by infiltration of foamy histocytes into the affected organs of different systems. ECD lesions are recognised to cause progressive scarring and fibrosis that pervade multiple organ...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-01-01
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| Series: | Rare |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2950008724000103 |
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| author | Qinglin Lau De Yee Gan Soon Ching Gan Nor Haisyah Binti Noor Kasim Ahmad Zakiyy Bin Mohamed |
| author_facet | Qinglin Lau De Yee Gan Soon Ching Gan Nor Haisyah Binti Noor Kasim Ahmad Zakiyy Bin Mohamed |
| author_sort | Qinglin Lau |
| collection | DOAJ |
| description | Erdheim-Chester disease (ECD) or lipoid granulomatosis is a rare non-langerhan cell histiocytosis disease characterised by infiltration of foamy histocytes into the affected organs of different systems. ECD lesions are recognised to cause progressive scarring and fibrosis that pervade multiple organs and systems. Being rare and notoriously insidious, ECD is often diagnosed late. In Malaysia, to date, there is only 1 case report pertaining to this rare disease in which the patient has unfortunately passed away just 4 months after diagnosis. In this case report, we acquired the opportunity to describe the multisystemic involvement of ECD in our patient. Furthermore, over the course of the 27-month endeavour, numerous obstacles have been identified, including our patient's less-than-ideal health-seeking behaviour as well as limitations with regard to imaging, laboratory modalities, and therapeutics. This case will highlight the unique challenges we overcame to solve this clinical conundrum in our publicly funded tertiary hospital in Malaysia. |
| format | Article |
| id | doaj-art-a13d0b0089b54ed094c3f20acd1ea5a2 |
| institution | Kabale University |
| issn | 2950-0087 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Rare |
| spelling | doaj-art-a13d0b0089b54ed094c3f20acd1ea5a22024-12-18T08:55:33ZengElsevierRare2950-00872024-01-012100027Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in MalaysiaQinglin Lau0De Yee Gan1Soon Ching Gan2Nor Haisyah Binti Noor Kasim3Ahmad Zakiyy Bin Mohamed4Department of Internal Medicine, Hospital Tengku Ampuan Rahimah, Ministry of Health, Selangor, Malaysia; Corresponding author.Department of Internal Medicine, Hospital Tengku Ampuan Rahimah, Ministry of Health, Selangor, MalaysiaDepartment of Pathology, Hospital Tengku Ampuan Rahimah, Ministry of Health, Selangor, MalaysiaDepartment of Imaging and diagnosis. Hospital Tengku Ampuan Rahimah, Ministry of Health, Selangor, MalaysiaHematology Unit, Hospital Tengku Ampuan Rahimah, Ministry of Health, Selangor, MalaysiaErdheim-Chester disease (ECD) or lipoid granulomatosis is a rare non-langerhan cell histiocytosis disease characterised by infiltration of foamy histocytes into the affected organs of different systems. ECD lesions are recognised to cause progressive scarring and fibrosis that pervade multiple organs and systems. Being rare and notoriously insidious, ECD is often diagnosed late. In Malaysia, to date, there is only 1 case report pertaining to this rare disease in which the patient has unfortunately passed away just 4 months after diagnosis. In this case report, we acquired the opportunity to describe the multisystemic involvement of ECD in our patient. Furthermore, over the course of the 27-month endeavour, numerous obstacles have been identified, including our patient's less-than-ideal health-seeking behaviour as well as limitations with regard to imaging, laboratory modalities, and therapeutics. This case will highlight the unique challenges we overcame to solve this clinical conundrum in our publicly funded tertiary hospital in Malaysia.http://www.sciencedirect.com/science/article/pii/S2950008724000103Erdheim-chester diseaseRetroperitoneal fibrosisHistiocytosisMultisystemicRare disease |
| spellingShingle | Qinglin Lau De Yee Gan Soon Ching Gan Nor Haisyah Binti Noor Kasim Ahmad Zakiyy Bin Mohamed Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia Rare Erdheim-chester disease Retroperitoneal fibrosis Histiocytosis Multisystemic Rare disease |
| title | Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia |
| title_full | Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia |
| title_fullStr | Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia |
| title_full_unstemmed | Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia |
| title_short | Erdheim-Chester disease: Challenges in diagnosing and treating a rare multisystemic disease in Malaysia |
| title_sort | erdheim chester disease challenges in diagnosing and treating a rare multisystemic disease in malaysia |
| topic | Erdheim-chester disease Retroperitoneal fibrosis Histiocytosis Multisystemic Rare disease |
| url | http://www.sciencedirect.com/science/article/pii/S2950008724000103 |
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