Proteus syndrome. a contribution to the further differential diagnosis of congenital local gigantism
Trappe, B.O.; Rupprecht, E.; Bosselmann, E.; Schaeper, M.; Mohnike, K.; Gedschold, J.
Helvetica Paediatrica Acta 43(5-6): 473-482
1989
ISSN/ISBN: 0018-022X PMID: 2745142 Document Number: 326921
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.