Archives of Craniofacial Surgery (대한두개안면성형외과학회지)

Korean Cleft Palate-Craniofacial Association (대한두개안면성형외과학회)
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A Case Report of Langerhans Cell Histiocytosis of Frontal Area

전두부에 발생한 랑게르한스세포 조직구증의 치험례

  • Yang, Hae Won (Department of Plastic and Reconstructive Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine) ;
  • Kang, Min Gu (Department of Plastic and Reconstructive Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine) ;
  • Chang, Choong Hyun (Department of Plastic and Reconstructive Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine)
  • 양해원 (성균관대학교 의과대학 강북삼성병원 성형외과학교실) ;
  • 강민구 (성균관대학교 의과대학 강북삼성병원 성형외과학교실) ;
  • 장충현 (성균관대학교 의과대학 강북삼성병원 성형외과학교실)
  • Received : 2008.08.21
  • Accepted : 2008.10.24
  • Published : 2009.04.09

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Abstract

Purpose: Langerhans cell histiocytosis is a heterogenous group of Langerhans cell proliferative disorders and includes eosinophilic granuloma, Letterer-Siwe diseases, and Hand-Schuller Christian disease. We report a case of eosinophilic granuloma on frontal area. Methods: A 17-year-old male presented with swelling and tenderness on Lt. frontal and periorbital area. CT and MRI showed a $33{\times}25mm$ sized mass that involved Lt. frontal calvarium, frontotemporal meninges, and orbital roof. Results: Total excision of the mass and adjacent soft tissue, calvarium, and orbital roof was performed. Orbital roof defect was reconstructed with absorbable plate and calvarial defect was done with outer cortex of temporal bone flap. The histology revealed proliferation of histiocytes and eosinophils. Immunologically, these histiocytic cells expressed S-100 protein and CD1a. The patient is currently taking conservative treatment. Conclusion: The severity of these disease and their prognosis and treatments are various. For unifocal cranial Langerhans cell histiocytosis, complete excision is the treatment of choice. We report this case with review of literature.

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