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.