Tuberculosis and Respiratory Diseases

  • 제67권4호
  • /
  • Pages.345-350
  • /
  • 2009
  • /
  • 1738-3536(pISSN)
  • /
  • 2005-6184(eISSN)
대한결핵및호흡기학회 (The Korean Academy of Tuberculosis and Respiratory Diseases)
권호 표지
DOI QR코드

DOI QR Code

Erdheim-Chester Disease with Hepatitis, Glomerulonephritis, Aplastic Anemia and Lung Involvement

  • Park, Ji Won (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Chung, Chae Uk (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Shin, Ji Young (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Jung, Sun Young (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Yoo, Su Jin (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Lee, Jeong Eun (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Jung, Sung Soo (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Kim, Ju Ock (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Kim, Sun Young (Department of Internal Medicine, College of Medicine, Chungnam National University) ;
  • Park, Hee Sun (Department of Internal Medicine, College of Medicine, Chungnam National University)
  • 투고 : 2009.05.21
  • 심사 : 2009.09.02
  • 발행 : 2009.10.30
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

Erdheim-Chester disease (ECD) is a proliferative non-Langerhans cell histiocytosis of multiple organs. This is a rare disease of unknown etiology with a high mortality. We present the case report of a 26-year-old man diagnosed with ECD. He was referred to our hospital with elevated levels of aminotransferases. Although the diagnosis was uncertain, the patient was lost to follow up at that time. One year later, the patient returned to the hospital with generalized edema. Although a specific bone lesion was not found, the patient was experiencing the following: glomerulonephritis, aplastic anemia, hepatitis, and lung involvement. A lung biopsy was performed: the immunohistochemical stain were positive for CD68 and negative for S-100 protein and CD1a. We diagnosed as the patient as havinf ECD. Approximately 50% of ECD cases present with extraskeletal involvement. ECD should be considered as part of the differential diagnosis when multiple organs are involved.

키워드

참고문헌

  1. Furmanczyk PS, Bruckner JD, Gillespy T 3rd, Rubin BP. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis. Skeletal Radiol 2007;36:885-9 https://doi.org/10.1007/s00256-007-0302-x
  2. Chung JH, Park MS, Shin DH, Choe KO, Kim SK, Chang J, et al. Pulmonary involvement in Erdheim-Chester disease. Respirology 2005;10:389-92 https://doi.org/10.1111/j.1440-1843.2005.00671.x
  3. Adem C, Helie O, Leveque C, Taillia H, Cordoliani YS. Case 78: Erdheim-Chester disease with central nervous system involvement. Radiology 2005;234:111-5 https://doi.org/10.1148/radiol.2341021806
  4. Al-Quran S, Reith J, Bradley J, Rimsza L. Erdheim-Chester disease: case report, PCR-based analysis of clonality, and review of literature. Mod Pathol 2002;15:666-72 https://doi.org/10.1038/modpathol.3880583
  5. Matsui K, Nagata Y, Hiraoka M. Radiotherapy for Erdheim-Chester disease. Int J Clin Oncol 2007;12:238-41 https://doi.org/10.1007/s10147-006-0644-8
  6. Kruger S, Krop C, Wibmer T, Pauls S, Mottaghy FM, Schumann C, et al. Erdheim-chester disease: a rare cause of interstitial lung disease. Med Klin (Munich) 2006;101:573-6 https://doi.org/10.1007/s00063-006-1068-y
  7. Spyridonidis TJ, Giannakenas C, Barla P, Apostolopoulos DJ. Erdheim-Chester disease: a rare syndrome with a characteristic bone scintigraphy pattern. Ann Nucl Med 2008;22:323-6 https://doi.org/10.1007/s12149-007-0110-3
  8. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M, et al. Erdheim-Chester disease: clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 1996;75:157-69 https://doi.org/10.1097/00005792-199605000-00005
  9. Lau WW, Chan E, Chan CW. Orbital involvement in Erdheim-Chester disease. Hong Kong Med J 2007;13:238-40
  10. Garg T, Chander R, Gupta T, Mendiratta V, Jain M. Erdheim-Chester disease with cutaneous features in an Indian patient. Skinmed 2008;7:103-6 https://doi.org/10.1111/j.1751-7125.2008.07372.x
  11. Loddenkemper K, Hoyer B, Loddenkemper C, Hermann KG, Rogalla P, Forster G, et al. A case of Erdheim-Chester disease initially mistaken for Ormond's disease. Nat Clin Pract Rheumatol 2008;4:50-5 https://doi.org/10.1038/ncprheum0677
  12. Dinkar AD, Spadigam A, Sahai S. Oral radiographic and clinico-pathologic presentation of Erdheim-Chester disease: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:e79-85 https://doi.org/10.1016/j.tripleo.2006.11.006
  13. Egan AJ, Boardman LA, Tazelaar HD, Swensen SJ, Jett JR, Yousem SA, et al. Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. Am J Surg Pathol 1999;23:17-26 https://doi.org/10.1097/00000478-199901000-00002
  14. Dickson BC, Pethe V, Chung CT, Howarth DJ, Bilbao JM, Fornasier VL, et al. Systemic Erdheim-Chester disease. Virchows Arch 2008;452:221-7 https://doi.org/10.1007/s00428-007-0538-9
  15. Yano S, Kobayashi K, Kato K, Tokuda Y, Ikeda T, Takeyama H. A case of Erdheim-Chester disease effectively treated by cyclophosphamide and prednisolone. Nihon Kokyuki Gakkai Zasshi 2007;45:43-8