Macrodactyly and proteus syndrome

Abstract

Macrodactyly is a rare congenital, nonhereditary malformation presenting as an increased size of one or several digits of the hands or feet. Proteus syndrome, consisting of various clinical manifestations, is a congenital hamartomatous syndrome involving many organ systems and characterized mainly by hemihypertrophy, macrodactyly, exostoses, pigmentary nevi and subcutaneous tumors. In rare cases, the clinical manifestations of Proteus syndrome may be limited to acral tissue hypertophy. We report a case with Proteus syndrome restricted macrodactyly of one hand. The differential diagnosis with macrodactyly and other hamartomatous overgrowth syndromes is discussed. Kaynaklar.