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Geroderma Osteodysplastica. Report of a New Family
Author(s) -
Boente María del Carmen,
Asial Raúl A.,
Winik Beatriz C.
Publication year - 2006
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 - 10.1111/j.1525-1470.2006.00285.x
Subject(s) - medicine , cutis laxa , differential diagnosis , dermatology , collagen fiber , osteoporosis , pathology , anatomy
We report a family in which geroderma osteodysplastica affected two male siblings. They showed the characteristic features associated with this syndrome: a prematurely aged face with wrinkly, lax skin, more prominent on the acral regions, associated with joint laxity, osteoporosis, and skeletal abnormalities. The main histologic abnormalities were fragmented elastic fibers that were diminished in number. Although collagen fibers showed changes in their orientation, they were normal in structure and number. We consider the differential diagnosis with other syndromes associated with cutis laxa using clinical, radiologic, and histopathologic criteria.