John Libbey Eurotext

European Journal of Dermatology

Focal dermal hypoplasia (Goltz-Gorlin syndrome) associated with obstructive papillomatosis of the larynx and hypopharynx Volume 9, numéro 8, December 1999


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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. [1] and Gorlin et al. [2]. 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 ...