A Case of Tuberous Sclerosis with Multiple Fibroma on Scalp and Extremity.
- Author:
Hyoung Suk KIM
1
;
Hii Sun JEONG
;
Keuk Shun SHIN
;
Sang Yeob LEE
;
Ji Sun SONG
Author Information
1. Department of Plastic and Reconstructive Surgery, College of Medicine, Kwandong University, Gyeonggi-do, Korea. drsks@kwandong.ac.kr
- Publication Type:Case Report
- Keywords:
Tuberous sclerosis;
Fibroma;
Tangential excision;
Dermabrasion
- MeSH:
Adult;
Angiofibroma;
Angiomyolipoma;
Astrocytoma;
Brain;
Dermabrasion;
Extremities;
Fibroma;
Forehead;
Hamartoma;
Heart;
Humans;
Intellectual Disability;
Kidney;
Lung;
Male;
Neurocutaneous Syndromes;
Recurrence;
Scalp;
Seizures;
Skin;
Tuberous Sclerosis
- From:Journal of the Korean Society of Plastic and Reconstructive Surgeons
2008;35(3):341-344
- CountryRepublic of 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.
