Gorham’s Disease of the Mandible

Gorham’s disease is a rare condition characterized by progressive osteolysis of bone with ultimate total disappearance of bone. The aetiology is undetermined. It may affect any bone of the body, although there is predilection for the pelvis, humerus, axial skeleton, and mandible. Because of the rarity, the disorder goes unrecognized. Various treatment modalities are undertaken with very limited success. We report a case of Gorham’s disease of mandible in a 62-year-old man and the literature is reviewed with emphasis on aetiology, diagnosis management. 1. Introduction Gorham’s Disease (GD) is a rare disorder characterized by destructive proliferation of vascular channels within the bone and the surrounding soft tissues. The first report of a case of boneless arm was in 1838 and again in 1872 by Jackson. Romer reported first case in jaws of the 13-year-old woman in 1924 [1–4]. The condition was named after two physicians, Lemuel whittington Gorham, MD, and Arthur Purdy Stout, MD. Various synonyms used in the literature to describe the condition include phantom bone, disappearing or vanishing bone disease, acute spontaneous absorption of bone, hemangiomatosis, lymphangiomatosis, idiopathic osteolysis and Gorham’s disease. The condition may affect any part of the skeleton, but most commonly involves the skull, shoulder, and pelvic girdle. About 50 cases of Gorham’s disease involving maxillofacial region are reported. Here we report a case of Gorham’s disease of the mandible and the literature is reviewed with emphasis on etiology, diagnosis and management. 2. Case Report A 62-year-old male patient reported to the outpatient department with chief complaint of mild discomfort and slight swelling in relation to lower left side of face. The patient gave a history of mobile tooth which was extracted 1 month back. Extra oral examination revealed mild diffuse swelling on lower left side of jaw (Figure 1). The overlying skin was normal. Intraoral examination revealed partially edentulous left mandible with missing molars which was extracted because of mobility (Figure 2). Orthopantomogram revealed an extensive ill-defined osteolytic lesion of body of mandible extending from 37 regions involving angle of mandible and extending up the ramus (Figure 3). Provisional diagnosis of intraosseous malignant neoplasm was made. Differential diagnosis included metastatic tumour, metabolic disease, and osteomyelitis. Hematological investigation of serum calcium, phosphorus, serum alkaline phosphatase and parathyroid hormone levels were found to be within normal range. The