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Wrinkly Skin Syndrome: Ultrastructural Alterations of the Elastic Fibers
Author(s) -
Boente María del C.,
Winik Beatriz C.,
Asial Raúl A.
Publication year - 1999
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.1999.00027.x
Subject(s) - ultrastructure , elastin , medicine , connective tissue , pathology , elastic fiber , anatomy , dorsum , differential diagnosis
We report the clinical and pathologic features of a patient with wrinkly skin syndrome. The essential clinical features were wrinkly skin with poor elasticity over the abdomen and on the dorsum of the hands and feet, increased palmar and plantar creases, and a prominent venous pattern over the chest. On light microscopy, histopathologic findings included appreciable heterogeneity in the structure, amount, and distribution pattern of elastic fibers. Agglutination and fragmentation of the microfibrillar component and a remarkable decrease in elastin were the major ultrastructural features. The differential diagnosis with other connective tissue disorders is discussed.