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Osteopoikilosis and Buschke-Ollendorff syndrome

Anna Kotulska, Eugeniusz J. Kucharz

CaseRepClinPractRev 2002; 3(4):290-293

ID: 474497

The disorder (known as spotted bones, diffuse osteopathy, osteopoikilosis) is a congenital disease of the skeletal system characterized by numerous, round foci of bone sclerosis localized within spongy bone tissue. Coincident
osteopoikilosis and cutaneous lesions (fibromas) is referred to as Buschke-Ollendorff syndrome. The disease is inherited as an autosomal dominant trait. Osteopoikilosis is usually asymptomatic and is diagnosed accidentally
in radiography performed for other reasons. Osteopoikilosis may be accompanied by melorheostosis and/or osteopathia striata. This syndrome is known as mixed sclerosing bone dystrophy.

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