Pachyonychia congenita

Abstract

Pachyonychia congenita represents a group of rare, autosomal dominant keratin disorders with characteristic nail findings, abnormalities of the palmoplantar skin, pilosebaceous apparatus, oral and laryngeal mucosa and teeth. The main clinical characteristic that gives the condition its name is the abnormally thickened nails. A case with typical clinical features is discussed.

Keywords

• Hyperkeratosis of nail bed
• leukokeratosis
• pachyonychia congenital