A Case of Tuberous Sclerosis with Multiple Fibroma on Scalp and Extremity

두피 및 사지에 다발성 섬유종을 동반한 결절성 경화증

  • Kim, Hyoung Suk (Department of Plastic and Reconstructive Surgery, College of Medicine, Kwandong University) ;
  • Jeong, Hii Sun (Department of Plastic and Reconstructive Surgery, College of Medicine, Kwandong University) ;
  • Shin, Keuk Shun (Department of Plastic and Reconstructive Surgery, College of Medicine, Kwandong University) ;
  • Lee, Sang Yeob (Department of Pathology, College of Medicine, Kwandong University) ;
  • Song, Ji Sun (Department of Pathology, College of Medicine, Kwandong University)
  • 김형석 (관동대학교 의과대학 성형외과학교실) ;
  • 정희선 (관동대학교 의과대학 성형외과학교실) ;
  • 신극선 (관동대학교 의과대학 성형외과학교실) ;
  • 이상엽 (관동대학교 의과대학 병리학교실) ;
  • 송지선 (관동대학교 의과대학 병리학교실)
  • Received : 2008.01.28
  • Published : 2008.05.10

Abstract

Purpose: Tuberous sclerosis is an autosomal dominant multisystemic neurocutaneous syndrome characterized by the development of multiple hamartoma distributed through the body, skin, brain, heart, kidney, and lung. The classic triad is seizure, mental retardation, and facial angiofibroma. We experienced a case of a tuberous sclerosis associated with the facial lesion and multiple masses on scalp, forehead, and right lower extremity. Methods: This a 34-year-old male patient had subependymal giant cell astrocytoma in brain and multiple angiomyolipoma in both kidneys. Tangential excision with razor blade and dermabrasion were done on the centrofacial area. We excised other lesions and the mass on scalp was excised and covered with split thickness skin graft. Results: The histopathological finding revealed that the facial lesion was angiofibroma and the others were multiple fibroma. Conclusion: In our case of tuberous sclerosis, we chose the tangential excision to remove the large nodules of angiofibroma, and then dermabrasion was used to smooth the final contour. The patient appeared to have a good results from this treatment modality. But, tuberous sclerosis is an disease that needs long term follow-up to check up the recurrence of skin problem.

Keywords

References

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