Oral manifestation of pachyonychia congenita type 1: Jadassohn lewandowsky syndrome

Pachyonychia congenital (PC), is a rare genetic disorder, autosomal dominant, disorder of keratinization. This condition characterized by cutaneous manifestation mainly hyperkeratosis skin and mucosae hypertrophy nails. In this condition, almost 50% the patients will have oral leukokeratosis. The case report here 15 years old girl, presented with dystrophic, thickened fingernails toenails subungual hyperkeratosis, palmoplantar keratoderma, hyperkeratotic plaques in buccal mucosae. Histological examination shows acanthosis, parakeratosis ballooning epithelial cells, these features were consistent leukokeratosis, has been diagnosed as Congenita type 1. hence, reported.