Geroderma osteodysplastica. Report of a new family.
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.
Boente
María del Carmen
Mdel C
Servicio de Dermatología, Hospital del Niño Jesús, Tucumán, Argentina. mboente@arnet.com.ar
Asial
Raúl A
RA
Winik
Beatriz C
BC
D001851:Bone Diseases, Metabolic / Q000235:genetics* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003483:Cutis Laxa / Q000235:genetics* / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D013577:Syndrome