Pachyonychia Congenita

**Definition:** A group of inherited ectodermal dysplasias whose most prominent clinical feature is hypertrophic nail dystrophy resulting in PACHYONYCHIA. Several specific subtypes of pachyonychia congenita have been associated with mutations in genes that encode KERATINS.

**Tree numbers:** - C16.131.077.350.856 - C16.131.831.350.856 - C16.320.850.250.856 - C17.800.529.594 - C17.800.804.350.856 - C17.800.827.250.856

**Synonyms:** - Congenital Pachyonychia - Pachyonychia Congenita Syndrome