Osteopoikilosis and the Buschke–Ollendorff syndrome

Abstract

Osteopoikilosis is an uncommon cause of multiple osteosclerotic bone lesions. The skeletal changes of osteopoikilosis (synonyms: osteopathia condensans disseminata, spotted bone) were first described by Stieda in 1905 and subsequently by Albers-Schoenberg (1915). Radiologically, the lesions consist of multiple wellcircumscribed round or oval opacities, each 1–10 mm in diameter. The mottling is more marked in the epiphyses and metaphyses of the long bones and the pelvis, but is also common in the spongiosa of the phalanges, carpal and tarsal bones. The changes are more common in men than women and usually present in adulthood, although osteopoikilosis has been reported in children. Familial aggregation has been described, and autosomal dominant inheritance with variable expression is proposed (Schoenenberg, 1975). Osteopoikilosis should be considered in the differential diagnosis of multiple osteosclerotic bone lesions, but is a relatively uncommon finding. Serowy (1956) could find only 100 reported cases in the literature over a 25-year period since its initial description, and Jonasch (1955) found only 12 cases of osteopoikilosis in a series of 21000 radiographs.