Wrinkly skin syndrome: Ultrastructural alterations of the elastic fibers

We report the clinical and pathologic features of a patient with wrinkly sk in syndrome. The essential clinical features were wrinkly skin with poor el asticity over the abdomen and on the dorsum of the hands and feet, increase d palmar and plantar creases, and a prominent venous pattern over the chest . On light microscopy, histopathologic findings included appreciable hetero geneity in the structure, amount, and distribution pattern of elastic fiber s. Agglutination and fragmentation of the microfibrillar component and a re markable decrease in elastin were the major ultrastructural features. The d ifferential diagnosis with other connective tissue disorders is discussed.