[Proteiform syndrome (Proteus syndrome). Skin manifestations in a recently observed case]

The case of a 4-year-old female affected by partial gigantism of the feet, syndactyly and polydactyly, partial right hemihypertrophy of buttock and lower limb, warty hyperpigmented nevus and vulvar lipoma is described. The Authors discuss about the Proteus syndrome, pointing out its rarity, the polymorphism and the problems of differential diagnosis with the Klippel-Trenaunay-Weber syndrome and with other congenital hamartomatous disorders.