Paget disease is a metabolic bone disease characterized by focal abnormalities of increased bone turnover affecting one or more sites throughout the skeleton, primarily the axial skeleton. Bone lesions in this disorder show evidence of increased osteoclastic bone resorption and disorganized bone structure. [From OMIM]
Hale and Kelly (1998) described an 82-year old female with Paget disease of bone and Kaposi's sarcoma. She was born to non-consangunieous parents. Seven of her siblings had previously been diagnosed with the condition. In addiiton, two of her siblings had been diagnosed wilh classic Kaposi's sarcoma.