Article Text

Download PDFPDF

Pachyonychia congenita
  1. W M El-Matary1,
  2. I Barnard2
  1. 1Neonatal Unit, Glan Clwyd Hospital, Rhyl, North Wales LL18 5UJ, UK
  2. 2CDC, Clatterbridge Hospital, Clatterbridge Road, Bebington, Wirral CH63 4JY, UK;

    Statistics from

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    We have recently experienced this rare condition in a premature baby born at 25 weeks gestation. At the age of 56 days, it was noticed that he had developed odd looking fingernails (two on each hand). He did not have any other mucocutaneous abnormalities or family history of similar conditions.

    A culture of his nails was reported as negative for bacterial and fungal infections, and the diagnosis of pachyonychia congenita was made on the basis of nail morphology.

    Pachyonychia congenita is a group of uncommon hereditary keratin disorders characterised by hypertrophic nail dystrophy and associated ectodermal features.1 However, it can be manifested with only nail affection.2 The nails are thickened and tubular, projecting upward at the free edge to form a conical roof over a mass of subungual keratotic debris (fig 1).

    Figure 1

     Thickened nail characteristic of pachyonychia congenita.


    View Abstract