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KMID : 0358820080350030341
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Journal of Korean Society of Plastic and Reconstructive Surgeons
2008 Volume.35 No. 3 p.341 ~ p.344
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A Case of Tuberous Sclerosis with Multiple Fibroma on Scalp and Extremity.
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Kim Hyoung-Suk
Jeong Hii-Sun
Shin Keuk-Shun
Yi Sang-Yeop
Song Ji-Sun
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Abstract
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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.
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KEYWORD
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Tuberous sclerosis, Fibroma, Tangential excision, Dermabrasion
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