Abstract
Pachyonychia congenita is a rare genodermatoses transmitted as an autosomal dominant trait with o nly 450 cases reported since 1906. It is of four types. Pachyonychia congenita type 1 is called as Jadassohn Lew andowsky syndrome.The syndrome results from mutations in the gene encoding epidermal keratins. The patients present with classical nail hypertrophy, palmoplantar hyperkeratosis and follicular keratotic papules on the bo dy. The case has been reported for its rarity and its rare association with metabolic syndrome.
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