Pachyonychia Congenita

Pachyonychia congenita is a rare and uncommon condition. It is quite possible, however that many cases are overlooked, and they may be diagnosed as a simple congenital deformity of the nails. Most of the cases reported have been in children, for the reason that this condition is a particular form of a congenital abnormality. This particular syndrome includes more than deformities of the finger nails. The title of Jadassohn and Lewandowsky's paper in 1906 "Keratosis Disseminata Circumscripta Tylomata and Keratosis Linguae" shows the essential features of this condition. Pachyonychia seems to be the constant symptom in all the cases reported. The others include bullae, palmar and plantar keratoses, leukoplakia, anomalies of the hair, follicular keratoses of an acneform type, especially on the knees and elbows, and dyskeratosis of the cornea. Heller1includes these changes under the term congenital dyskeratoses. Most of the cases seem to have been reported in males