Gorham's disease: A diagnostic challenge

Abstract

Gorham's disease is a rare disorder of uncertain etiology characterized by spontaneous and progressive osteolysis of one or more skeletal bones. Till date, less than 200 cases have been reported in the international literature with about 51 cases involving the maxillofacial site. The radiographic findings associated with Gorham's disease are particularly dramatic, as in some cases a complete resorption of the involved bone can occur, leading to the definition of phantom bone or disappearing bone disease. The purpose of this review is to make our community aware of this rare entity and to discuss the etiopathology, clinical presentation, radiographic findings, histopathology, differential diagnoses and treatment modalities for patients with vanishing bone disease.

Keywords: Gorham's disease; mandible; massive osteolysis; vanishing bone disease.

Figure 1

Radiographic image shows radiolucency in 35, 36, 37 and38 region with their displacement and root resorption. The teeth show no bony support. Right side of mandible appears normal (Courtesy: Dong Q, Zhang Y,Sun C, Guo J. Gorham-Stout syndrome affecting the left mandible: A case report.J Exp Therapeut Med 2012:162-4)

Figure 2

Histopathological image shows vascular proliferation intermixed with fi brous connective tissue and a chronic infl ammatory cell infi ltrate with thinning of bony trabeculae (H&E stain, ×100). (courtesy: www.tumorlibrary.com/case/detail.jsp?image_id=1629)