[Proteus syndrome. A contribution to the further differential diagnosis of congenital local gigantism].

Abstract

Four sporadically occurring characteristic cases of the Proteus syndrome are described. All patients show the main features of the disease, consisting of hemihypertrophy, partial gigantism of hands and/or feet with soft-tissue hypertrophy, plane naevi, subcutaneous tumours, and cranial anomalies. There is a remarkable polymorphism of the symptoms. Anamnestic, clinical and paraclinical findings are shown in tables. Based on a survey of the literature, the main aspects of genetics and of differential diagnosis are discussed.