ABSTRACT

Observations:

Pachyonychia congenita is an autosomal dominant genodermatosis characterized by subungual hyperkeratosis of the distal n ails and focal palmoplantar hyperkeratosis. It is classified as pachyonychia congenita type 1 (Jadassoh n-Lewandowsky) and pa chyonychia cong enita type 2 (J ackson- Lawler) s yndrome s. Herein a case of Pachyo nych ia conge nita type 1 i n a m iddle ag ed Kashmiri female is reported in view of the clinical rarity of this condition.