Gorham's disease of the maxilla – A rare case report with literature overview

Abstract

Gorham's disease is a rare condition characterized by progressive osteolysis of bone ultimately resulting in the total disappearance of bone. The etiology is unknown and the disease predominantly affects the pelvis, humerus, and axial skeleton. Because of its unusual, ambiguous presentation and rare occurrence, the disorder goes unrecognized and is often masqueraded by other disorders. The diagnosis of this disorder is by exclusion. About 50 cases of Gorham's disease involving the maxillofacial region are reported to date and most of them involve the mandible. Exclusive involvement of maxilla is documented in only 4 cases. We report an exceptional case of Gorham's disease of the maxilla in a 68-year-old male patient presenting with chronic pain and masquerading as an odontogenic infection.

The molecular mechanism of Gorham syndrome: an update

“…The osteolysis can be terminated by itself. At present, there is no specific index for Gorham syndrome diagnosis, which is an exclusive diagnosis that primarily depends on a combination of pathological and imaging examinations (7). In 1983, Heffez et al proposed the following eight diagnostic criteria for osteolysis (8): a positive bone biopsy; little or no osteoblast response; no malnourished calcification; non-dilated non-ulcerative lesions; no visceral involvement; an osteolytic imaging model; local progressive bone resorption; no hereditary, metabolic, neoplastic, immunological, or infectious venereal etiology; and no cellular atypia.…”