Aryal E*, Bastakoti S, Bhatarria S, Shrestha SB and Pokhrel G
Department of Dermatology, Kathmandu Medical College Teaching Hospital, NepalFulltext PDF
Introduction: Tuberous Sclerosis Complex (TSC) is a multisystem disorder characterized by growth of benign tumors in various organs of the body, caused by mutations of TSC1 or TSC2 gene. Periungual fibromas are a common cutaneous manifestation and a frequent source of cosmetic and functional concern. The purpose of this paper is to report a rare case of Koenen tumours with 20 nail involvement.Case Report: A housewife aged 55 years presented with complaint of difficulty in doing regular household work due to periungal growths on both hands. She had history of 2-3 episodes of seizures during childhood. Physical examination revealed firm, smooth, skin coloured periungal growths in all 20 nails suggesting ‘Koenen tumors’. Multiple facial angiofibromas and ash leaf macules on the back, were seen. We diagnosed the case as TSC on the basis of above findings which fulfilled three major diagnostic criterias. Koenen tumors of bilateral thumbs were ablated by CO2 laser.Discussion: TSC is a lifelong condition. Symptoms may cause disfigurement and disability, as in this case. So, early diagnosis and regular monitoring is very essential. Till date, a case of Koenen tumors involving 20 nails has not been reported from Nepal.
Tubereous sclerosis complex; Koenen tumors; 20 Nail
Aryal E, Bastakoti S, Bhatarria S, Shrestha SB, Pokhrel G. Koenen Tumors 20 Nail Involvement: A Rare Case Report. Ann Clin Case Rep. 2017; 2: 1411.