JD 1999:26
677-681
Multiplex
Pachyonchia
congenita (PC) is an uncommon autosomal dominant genodermatosis affecting the nails
and other ectodermal tissues. The most striking features are symmetrically thickened
dysmorphic nails and hyperkeratotic skin lesions. We report a case of pachyonychia
congenita in a 30-year-old male patient who had thickening and gray-brown discoloration
of all nails and many nodules on his back and neck. He also had hyperkeratotic skin
lesions on both feet. His tongue had irregularly-shaped, whitish plaques. Histology
of these nodules revealed the characteristic features of steatocystoma multiplex.
After treatment with oral retinoic acid, his hyperkeratotic skin lesions improved. |
