Cowden's disease – a rare case.

Abstract

Cowden’s disease is an autosomal dominant genetic syndrome characterized by multiple hamartomas involving organs derived from all three germinal layers. Incidence of Cowden’s disease is estimated at approximately 1:2,00,000. A 24 year female presented with multiple asymptomatic skin lesions over the face, of 18 months duration. Mucosa of oral cavity showed firm verrucous lesions and cobble stone appearance. On skin biopsy Trichilemmoma was diagnosed. Oral mucosal biopsy was diagnosed as fibroepithelial polyp. Correlating with the clinicopathological features, a final diagnosis of Cowden’s disease was made.