Buschke-Ollendorf Syndrome

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Abstract
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.