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

Kim, Hyoung Suk; Jeong, Hii Sun; Shin, Keuk Shun; Lee, Sang Yeob; Song, Ji Sun

Case Report

Journal of the Korean Society of Plastic and Reconstructive Surgeons 2008;35(3):341-344.
Published online May 1, 2008.

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

Hyoung Suk Kim, Hii Sun Jeong, Keuk Shun Shin, Sang Yeob Lee, Ji Sun Song

¹Department of Plastic and Reconstructive Surgery, College of Medicine, Kwandong University, Gyeonggi-do, Korea. drsks@kwandong.ac.kr
²Department of Pathology, College of Medicine, Kwandong University, Gyeonggi-do, Korea.

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: Tuberous sclerosis; Fibroma; Tangential excision; Dermabrasion