Buschke-Ollendorf syndrome. An electron microscopic study.
Two identical twins with the Buschke-Ollendorf syndrome (dermatofibrosis lenticularis disseminata with osteopoikilosis) are described. Ultrastructurally elastic fibers show clumps of electron-lucent elastin material coated with fine fibrils. The fibroblasts show enlarged endoplasmic reticulum containing abnormal fibrillar material. Juvenile elastoma seems to be a better term than dermatofibrosis. The pathogenesis of the elastic defect is discussed.
D002648:Child; D004200:Diseases in Twins; D004547:Elastic Tissue / Q000648:ultrastructure*; D005350:Fibroma / Q000473:pathology*; D006801:Humans; D008854:Microscopy, Electron; D010023:Osteopoikilosis / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D010026:Osteosclerosis / Q000473:pathology*; D011859:Radiography; D012878:Skin Neoplasms / Q000473:pathology*; D013577:Syndrome