Polidisplasia meso-ectodermica con ipoplasia dermica focale (Sindrome di Goltz ). Presentazione di un caso.

The relevant literature, clinical picture, aetiopathogenesis and differential diagnosis of meso ectodermic polydysplasia with focal dermic hypoplasia are described and a personal case observed in a male subject is presented. This is unusual, since the disease, which is probably of genetic origin, is apparently transmitted with dominance linked to the X chromosome and XY mortality. The subject presented skin atrophy, molluscoid pseudo tumours, colobomas of the iris and choroid, left palpebral ptosis, upper lip papillomatosis, foot syndactylies, and alterations of the nails. His histological picture showed typical skin changes, marked by a virtual absence of derma. The subcutaneous adipose tissue was in direct contact with a thin and linear epidermis.