Geroderma osteodysplastica. Report of a new family

BOENTE MC; ASIAL RA; WINIK BC

PEDIATRIC DERMATOLOGY

Año: 2006 vol. 23 p. 467 - 472

0736-8046

Abstract: We report a family in which geroderma osteodysplastica affected two male siblings. They showed the characteristic features associ-ated 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 frag-mented 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.We report a family in which geroderma osteodysplastica affected two male siblings. They showed the characteristic features associ-ated 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 frag-mented 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.