Tuberous sclerosis: report of a case with fibromatosis of the facial soft tissues.
Tuberous Sclerosis (TS) is a dominantly inherited disorder which is characterised by a triad of mental deficiency, epilepsy and angiofibromas of the face. Sub- and periungual fibromas, shagreen patches usually found in the lumbosacral region and hypopigmented skin lesions are less often seen. Paraventricular calcifications, skeletal disorders and rarely, ophthalmic tumours are also described. Oral manifestations include whitish confluent nodules on the mucosa, enamel hypoplasia, mandibular cysts, and oral fibromas. A case of fibromatosis of the subcutaneous tissue overlying the angle of the mandible is described together with TS. To the best knowledge of the authors, this is the first such case reported in the literature.
Weber
A
A
Department of Oral Pathology and Oral Biology, University of Pretoria.
Ligthelm
A J
AJ
Scheepers
A
A
D002675:Child, Preschool; D005350:Fibroma; D006801:Humans; D008297:Male; D008339:Mandibular Neoplasms; D014402:Tuberous Sclerosis