안와 주위 봉와직염을 동반한 랑게르한스 세포 조직구증 1례

A Case of Langerhans Cell Histiocytosis Mimicking Periorbital Cellulitis

  • 유하연 (연세대학교 의과대학 소아과학교실) ;
  • 김기환 (연세대학교 의과대학 소아과학교실) ;
  • 최준정 (연세대학교 의과대학 병리학교실) ;
  • 김동수 (연세대학교 의과대학 소아과학교실)
  • Yoo, Ha Yeon (Department of Pediatrics, Yonsei University College of Medicine) ;
  • Kim, Ki Hwan (Department of Pediatrics, Yonsei University College of Medicine) ;
  • Choi, Jun Jeong (Department of Pathology, Yonsei University College of Medicine) ;
  • Kim, Dong Soo (Department of Pediatrics, Yonsei University College of Medicine)
  • 투고 : 2009.07.13
  • 심사 : 2009.09.25
  • 발행 : 2009.12.25

초록

랑게르한스 세포 조직구증은 소아에서 매우 드믄 질환이지만 발병시 경과나 증세가 매우 다양하게 나타날 수 있으며 다른 염증성 질환으로 오인될 소지가 있다. 따라서 임상 의사들은 이 병의 특징이나 경과에 대해 잘 알고 있어 병의 진단이나 치료가 늦어지지 않도록 주의하여야 하겠다. 저자들은 최근 안와주위 부종과 열감을 주소로 내원한 1세 남아에게서 조직 검사상 랑게르한스 세포 조직구증이 진단 되었기에 문헌고찰과 함께 보고하는 바이다. 항생제 치료 수일 후에도 호전이 없는 안와 염증의 경우 랑게르한스 세포 조직구증을 반드시 감별 질환의 하나로 고려해야 한다.

Langerhans cell histiocytosis is a rare disease in children. However, Langerhans cell histiocytosis encompasses a wide spectrum of clinical presentations and mimics other conditions. A 1-year-old boy presented with signs of periorbital cellulitis that initially responded to antibiotics, but remained as a same-sized mass with serial orbital computed tomography. The lesion was partially excised. Histopathology and immunohistochemical staining confirmed the diagnosis of Langerhans cell histiocytosis. This case demonstrates that in patients with periorbital cellulitis which has relapsed or responded inadequately to antibiotics, further investigation should initiated to rule out other inflammatory causes.

키워드

참고문헌

  1. Howe L, Jones NS. Guidelines for the management of periorbital cellulitis/abscess. Clin Otolaryngol Allied Sci 2004;29:725-8. https://doi.org/10.1111/j.1365-2273.2004.00889.x
  2. Rimon A, Hoffer V, Prais D, Harel L, Amir J. Periorbital cellulitis in the era of haemophilus influenzae type b vaccine: Predisposing factors and etiologic agents in hospitalized children. J Pediatr Ophthalmol Strabismus 2008;45:300-4. https://doi.org/10.3928/01913913-20080901-14
  3. Erly WK, Carmody RF, Dryden RM. Orbital histiocytosis x. AJNR Am J Neuroradiol 1995;16:1258-61.
  4. Moore AT, Pritchard J, Taylor DS. Histiocytosis x. An ophthalmological review. Br J Ophthalmol 1985;69:7-14. https://doi.org/10.1136/bjo.69.1.7
  5. Rootman J. Diseases of the orbit : A multidisciplinary approach, 2nd ed. Philadelphia: Lippincott Williams & Wilkins, 2003;409-11.
  6. Margo CE, Goldman DR. Langerhans cell histiocytosis. Surv Ophthalmol 2008;53:332-58. https://doi.org/10.1016/j.survophthal.2008.04.007
  7. Yi G, Yoon HK, Kim BK, Kim KA, Choo IW. CT findings of orbital langerhans cell histiocytosis. J Korean Radiol Soc 2000;42:841-6. https://doi.org/10.3348/jkrs.2000.42.5.841
  8. Koo YH, Kim HK, Ahn YB, Lee SK, Park MS. Two cases of Langerhans' cell histiocytosis: Report of two cases. J Korean Neurosurg Soc 1997;26:430-4.
  9. Bhatti MT. Orbital syndromes. Semin Neurol 2007;27: 269-87. https://doi.org/10.1055/s-2007-979685
  10. Lichtenstein L. Histiocytosis X; integration of eosinophilic granuloma of bone, Letterer-Siwe disease, and Schuller- Christian disease as related manifestations of a single nosologic entity. AMA Arch Pathol 1953;56: 84-102.
  11. Kapur R, Sepahdari AR, Mafee MF, Putterman AM, Aakalu V, Wendel LJ et al. MR imaging of orbital inflammatory syndrome, orbital cellulitis, and orbital lymphoid lesions: The role of diffusion-weighted imaging. AJNR Am J Neuroradiol 2009;30:64-70. https://doi.org/10.3174/ajnr.A1494
  12. Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970;80:1414-28. https://doi.org/10.1288/00005537-197009000-00007
  13. Coenraad S, Buwalda J. Surgical or medical management of subperiosteal orbital abscess in children: A critical appraisal of the literature. Rhinology 2009;47:18-23.
  14. Hirsch M, Lifshitz T. Computerized tomography in the diagnosis and treatment of orbital cellulitis. Pediatr Radiol 1988;18:302-5. https://doi.org/10.1007/BF02388996
  15. Feldman RB, Moore DM, Hood CI, Hiles DA, Romano PE. Solitary eosinophilic granuloma of the lateral orbital wall. Am J Ophthalmol 1985;100:318-23. https://doi.org/10.1016/0002-9394(85)90800-1
  16. Nakajima A, Abe T, Takagi T, Satoh N, Sakuragi S, Miura I et al. Two cases of malignant lymphoma complicated by hemophagocytosis resembling orbital cellulitis. Jpn J Ophthalmol 1997;41:186-91. https://doi.org/10.1016/S0021-5155(97)00036-1
  17. Levy J, Monos T, Kapelushnik J, Maor E, Nash M, Lifshitz T. Langerhans cell histiocytosis with periorbital cellulitis. Am J Ophthalmol 2003;136:939-42. https://doi.org/10.1016/S0002-9394(03)00487-2