The Cutaneous Features Of Tuberous Sclerosis : A Hospital Based Study In South India

Abstract

Tuberous sclerosis is now frequently designated as the tuberous sclerosis complex (TSC) Its inheritance is autosomal dominant, but upto 70% of the cases are new mutations. Its prevalence was found to be 4.50 per 10.000 cases attending our dermatology outpatient department. A total of 9 patients attended the out patient department between August 1998 to June 2000. Five were males and 4 females. Eight cases were having definite TSC, whereas a single had suspect TSC. The age of our patients ranged from 10 months to 50 years. A positive family history was available in 4 cases. Most common cutaneous manifestation of TSC was adenoma sebaceum (8 cases) followed by shagreen patches (7 cases) and Koenen’s tumour (6 cases). Ash leaf macules and forehead plaques were seen in 5 cases each. Other findings were skin tags (3 cases), café-au-lait macules(2 cases), enamel pits (2 cases), portwine stain (1 case) and subconjunctival naevus (1 case). Classical triad of epilepsy, mental retardation and adenoma sebaceum was seen in one case only.