Orthopedics - Oleksa A.P. 2006

Pathophysiology of Joints
Developmental Defects and Congenital Skeletal Anomalies
Systemic Ossifying Periostoses

Systemic ossifying periostosis was first described in 1980 by Pierre Marie and Bamberger simultaneously, which is why it is referred to as Marie–Bamberger disease. In literature, it is also known under the names generalized hyperplastic periostitis, generalized ossifying periostosis, and generalized osteophytosis.

According to S.A. Reinberg (1964), this disease is not always generalized nor is it an inflammatory process; rather, it consistently exhibits a multiple systemic character and represents an ossifying periosteal process rather than a hyperplastic one. The primary Features of the syndrome are considered to be:

1) clubbed fingers and watch-Glass Nails;

2) chronic inflammation of the periosteum with Bone tissue apposition in the distal parts of long bones;

3) joint inflammation.

The exact cause of the disease remains unclear to this day. Systemic ossifying periostosis occurs in association with oncological, tuberculous, and purulent processes in the Lungs, bronchial adenitis, Pleurisy, and others. Therefore, when ossifying periostosis is detected, the patient's lungs should be examined, as it manifests within 1–1.5 months in cases of malignant Lung Tumors. Ossifying periostosis is also observed in Heart and mediastinal diseases.

B.M. Sosina and N.P. Zolotarska (1955) believe that the key factor is not The Nature of the pulmonary or cardiac disease, but rather its localization. Specifically, the localization of any process within the Mediastinum can serve as a reflexogenic zone for secondary bone reactions.

Sometimes, systemic periostosis is accompanied by digital clubbing (in heart disease) with nail deformation, thickening of the Skin—especially on the face and Nose—and skin pigmentation.

Among the pathogenetic factors, one can distinguish: 1) the action of toxins generated in the primary focus; 2) inadequate arterial Blood oxygen saturation; 3) local impairment of Blood Circulation and oxygenation; 4) hypothyroidism; 5) The production of substances that stimulate osteoblasts.

Hyperperiostosis most frequently occurs in young individuals, whose periosteum is most active even under normal conditions. The course of the disease is very slow, spanning years, but in growing children, this process develops more rapidly and tends to be symmetrical.

Patients most commonly complain of aching pain in the BONES OF THE hands and feet, and less frequently in other areas. Objective Examination of the affected bone regions reveals no significant changes. The joints are normal, and their function is preserved.

The primary diagnostic tool is X-ray Examination. Since the peripheral tubular bones are most commonly affected, the most characteristic changes are visible on radiographs of the metacarpal and Metatarsal Bones. As a rule, periosteal bone apposition in the form of periostosis is detected in the diaphyses and partially in the metaphyses, uniformly encasing the bones. A characteristic feature is the simultaneous involvement of the diaphyses of all Metacarpal bones and Phalanges without pathological Changes in the endosteum and without narrowing of the bone cavities. Furthermore, the terminal phalanges are spared, although clinical examination sometimes reveals clubbing and thinning of the terminal bone phalanges.

Initially, periostosis is clearly visible against the Background of the dense cortical bone layer, after which it gradually merges with it into a solid mass, thickening the bone. The structural pattern of the bone remains unchanged.

S.A. Reinberg points out that early-stage periostosis can resolve and disappear once the underlying causative process in the lungs is eliminated. Such resorption occurs quite rapidly, with a simultaneous disappearance of pain.