Synonym: Osteodystrophy deforman
Definition and clinical picture
Paget's disease is a chronic inflammatory progressive skeletal disorder mainly occurring after the age of 40. Men are more frequently affected than women. It is characterised by increased bone remodelling weakening the bone structure with enlargement and deformities of the affected bones. In 80% of the cases, the disorder is polyostotic, with the vertebral column, pelvis and skull being most commonly affected. It occurs relatively frequently in Northern Europe and the British Isles, and is extremely rare in China and Japan. Not uncommonly, it may occur in several members of an individual family. Its aetiology remains unclear; genetic component (locus on chromosome 18q) and viral (paramyxovirus) causes have been implicated.
Clinical symptoms: headache, bowing and shortening of the legs, the arms appear relatively long, enlargement of the head (hat and dentures no longer fit), and in extreme cases a leontine facies develops (leontiasis ossea). The patient's overall stature becomes smaller and monkey-like. Constriction around the origin of nerves may result in neuralgic symptoms and paraesthesia, or even blindness and deafness. The monostotic form shows individual foci in both the maxilla and the mandible. The maxillary sinus may be partially or completely filled with new bone. Progeny may occur in the mandible. The alveolar processes are enlarged. Changed tooth positions may result in occlusal disorders. Hypercementosis often develops around the tooth roots.
Proof - Histopathological findings
Radiograph: Mixed lucencies and opacities producing the classic cotton woll appearance. Diffuse hyperostosis with circumscribed osteoporotic changes occurs in the skull.
Scintigraphy: Increased perfusion and vascularisation, possibly simulating a tumour.
Laboratory: Raised levels of serum alkaline phosphatase.
Histology: Evidence of bone resorption and production with proliferation of large osteoclasts and osteoblasts, development of absorption lacunas, osteoid and newly formed trabeculae with lamellar maturation. Numerous unstructured cement lines and ground substance (mosaic structure) in enlarged bone trabeculae.
Fibrous dysplasia, cemento-osseous dysplasias, chronic suppurative osteomyelitis, osteosarcom
Treatment and prognosis
There is no causal treatment.
Osteotomies may be possible. Administration of calcitonin and vitamin D.
After a period of 10 - 20 years, malignant transformation and development of osteosarcoma may occur in approx. 3 - 30% of cases, and more rarely chondrosarcoma or fibrosarcoma (Paget's sarcoma).
- Paget J (1877) On a form of chronic inflammation of bones (osteitis deformans) Medico-Chirurgical Transactions, London, 1877, 60: 37-63
- Peters KM (Hrsg.) (2002) Knochenkrankheiten