Universitäts-Kinderklinik, Mathildenstraße 1, 79106 Freiburg, Germany.
- Page(s) : 618-20
- Année de parution : 1999
Focal dermal hypoplasia (FDH) is a rare syndrome associated with cutaneous,
skeletal, dental, ocular and soft-tissue defects. It was first described
independently by Goltz et al.  and Gorlin et al. .
The pathogenic mechanism is still illusive. An X-linked dominant mode
of inheritance with early intrauterine death of male subjects has been
assumed [3, 4]. A striking feature of FDH is the occurrence of giant papillomas