Buschke-Ollendorff Syndrome(A Case Report)

Abstract

The osteopoikilosis is commonly known as harmatoma in which metaphyseal and epiphyseal area of long bones and the bone of pelvis, hands, feet and et al, contain islands of dense cortical bone with normal Harversian system without any symptoms. The radiologic findings of the osteopoikilosis is multitude of oval or well-circumscribed areas of increased density, 2 to 10 mm in size, in symmetrical distribution and normal uptake in bone scan. Buschke-Ollendorff syndrome, which is transmitted by autosomal dominant, is characterized by the association of osteopoikilosis and connective tissue nevi which are popular and symmetrically distributed lesions on chest, back, buttock, thigh or arm. Authors report three Buschke-Ollendorff syndrome, studied by CT scan, MRI, bone scan and bone and skin biopsy, among five patients associated with osteopoikilosis found by simple radiologic study from ten members in one family with their pedigree.