ABSTRACT

The focal dermal hypoplasia is a X- dependend dominant cangenital disorder characterized by ectodermal and mesodermal malformations of the skin and its appendages. The characterized features; of the disease are on the skin lineer or reticular brownish red, some ofwhich telengiectasic atrophic lesions, anetoderma-like hemies, scars, on the mucoses papillomas, crab - claw deformity, shortness, osteopathia striata appearence on the metaphysis of long bones. We are presented a 30 year-old woman with the focal dermal hypoplasia.