Pachyonychia congenita: A case report

Abstract

Pachyonychia congenita (PC) is a rare autosomal dominant disorder of keratinization. It is usually present at birth or develops within 1 year of birth. It is classified into four types, but type-1 (Jadassohn–Lewandowsky type) and type-2 (Jackson–Lawler type) are the most common. PC is characterized by dystrophic, thickened nails, and painful palmo–plantar keratoderma. The nails are dramatically affected in most patients, but oral lesions are seen in patients affected by the Jadassohn–Lewandowsky type. In literature, fewer than 500 cases have been reported about PC. We report a case of PC in an 18-year-old male patient who presented with subungual hyperkeratosis, palmar-plantar keratosis, and leukokeratosis.