gorham 's disease also called as vanishing bone disease , acute spontaneous absorption of bone , hemangiomatosis , lymphangiomatosis , is a rare disease characterized by proliferation of thin - walled vascular channels which leads to destruction and resorption of osseous matrix . numerous names have been used in the literature [ table 1 ] . synonyms used for gorham 's disease idiopathic osteolysis was first described in 1838 by jackson as a case of disappearing humerus and led him title his work as  a boneless arm. it was romer in 1924 who reported first case in jaws . it was gorham in 1954 and gorham and stout in 1955 who presented a case series . the condition was named after these physicians , lemuel whittington gorham and arthur purdy stout as gorham and stout syndrome . since the initial description of this disease , 60 years have elapsed ; but the etiology of gorham 's disease is still very speculative . there is no evidence of a malignant , neuropathic , or infectious component involved in the causation of this disorder . the pathologic process is the replacement of normal bone by an aggressively expanding nonneoplastic vascular tissue similar to a hemangioma or lymphangioma . it is likely that the blood flow through these vessels is slow , which produces local hypoxia and lowering of the ph , favoring the activity of various hydrolytic enzymes . strong activity of acid phosphatase and leucine aminopeptidase in mononuclear perivascular cells which are in contact with bone indicates that these cells are important in the process of osseous resorption . gorham and stout hypothesized that trauma may trigger the process by stimulating the production of vascular granulation tissue and that  osteoclastosis  is not necessary . , have suggested that bone resorption in patients with gorham disease is due to enhanced osteoclast activity and interleukin 6 plays a role in the increased resorption of bone . evidence that osteolysis is due to an increased number of stimulated osteoclasts was presented by moller et al . , cellular and humoral mechanisms of osteoclast formation and bone resorption was reported by hirayama et al . , which suggested that the increase in osteoclast is not due to an increase in the number of circulating osteoclast precursors , but rather is due to an increase in the sensitivity of these precursors to humoral factors , which promote osteoclast formation and bone resorption . it has also been suggested that thyroid c cells and calcitonin may play an important role in the pathogenesis of gorham 's disease . gorham 's disease is a rare condition of osteolysis without sex , race , or age predilection ; affecting bones in different regions . based on clinical , histological and radiological features ; diagnosis is difficult and requires exclusion of neoplastic , inflammatory , infectious and endocrinologic disease . disappearing bone disease can be severely disabling and about 16% of cases are fatal . it is usually monocentric , but locally aggressive with resorption of the affected bone extending into adjacent soft tissues . many investigators believe that massive osteolysis is an aggressive form of skeletal angiomatosis that is related to vascular proliferation and shows regional involvement , frequently involving the shoulder and hip areas and has been termed as hemangiomatosis of bone . massive osteolysis has been documented in patients upto 70 years of age , but most affected patients are children and young adults . the most typical presentation is that of osteolysis of a single bone or the bones connected by a shared joint , such as the shoulder . shoulder is one of the most commonly involved areas , along with the skull and pelvic girdle . lack of bone healing following fracture is the hallmark of the disease . in 30% of affected patients , maxillofacial involvement is noted , with the mandible being the most frequently affected jaw bone . sign and symptoms include mobile teeth , pain , malocclusion , deviation of mandible and clinically obvious facial deformity . obstructive sleep apnea syndrome has been noted secondary to posterior mandibular displacement after extensive osteolysis . involvement of temporomandibular joint ( tmj ) by gorham disease can be mistaken for tmj dysfunction . although the degree of osseous deformity in patients with massive osteolysis may become severe , serious complications are infrequent . paraplegia related to spinal cord involvement may occur in patients who have involvement of vertebrae . thoracic cage , pulmonary or pleural involvement can lead to compromise of respiratory function and death can ensue . heffeze et al . , described the criteria for diagnosis of massive osteolysis as : 
 minimal or no osteoblastic response and absence of dystrophic calcification;positive biopsy for angiomatous tissue;absence of cellular atypia;evidence of local progressive osseous resorption;non - expansile , non - ulcerative lesion;absence of visceral involvement;osteolytic radiographic pattern;negative hereditary , metabolic , neoplastic , immunologic or infectious etiology . minimal or no osteoblastic response and absence of dystrophic calcification ; positive biopsy for angiomatous tissue ; absence of cellular atypia ; evidence of local progressive osseous resorption ; non - expansile , non - ulcerative lesion ; absence of visceral involvement ; osteolytic radiographic pattern ; negative hereditary , metabolic , neoplastic , immunologic or infectious etiology . anavi et al . , reviewed that the mean duration of symptoms before diagnosis was 6.4 years with the initial histopathological diagnosis inaccurate in 45% of cases , which highlights the difficulty faced by the clinician and the pathologists when attempting to diagnose the condition . the standard laboratory tests are usually within normal limits and do not help in diagnosis of massive osteolysis . radiographs , computed tomography ( ct ) , and magnetic resonance imaging ( mri ) have all been used . during the initial stage of the lesion , radiolucent foci appear in the intramedullary or subcortical regions , resembling findings seen in patchy osteoporosis . subsequently , slowly progressive atrophy , dissolution , fracture , fragmentation and disappearance of a portion of the bone occur with tapering or  pointing  of the remaining osseous tissue and atrophy of soft tissues . panoramic radiograph shows resorption and decreased vertical height of the mandibular body with the resorption extended towards the basal bone 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 ) radioisotope bone scan may show increased vascularity on initial images and subsequently , an area of decreased uptake corresponding to the site of diminished or absent osseous tissue . the microscopic findings contrast sharply with the striking clinical and radiographic findings . in the early stages , tissue removed from radiolucent defects consists of a nonspecific vascular proliferation intermixed with fibrous connective tissue and a chronic inflammatory cell infiltrate composed of lymphocytes and plasma cells . osteoclastic reaction in adjacent bone fragments is usually not conspicuous . according to johnson and mcclure there are two stages : the first with vascular proliferation , followed by a second stage in which residual fibrous tissue replaces resorbed bone [ 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 ) . the medical treatment for gorham 's disease includes radiation therapy , antiosteoclastic medications ( bisphosphonates ) , and alpha-2b interferon . surgical options include resection of the lesion and reconstruction using bone grafts and/or prostheses . in most cases , unfortunately , no single treatment modality has proven effective in arresting the disease . in patients who receive high - dose radiation therapy , the potential for secondary malignancy and growth restriction exists and the diagnosis of gorham 's disease should be suspected or made only after excluding other common underlying causes of osteolysis . aneurysmal bone cyst , extensive metastatic bone disease and osteosarcoma are some of the lesions that resemble vanishing bone disease and can be confirmed with a biopsy . when gorham 's disease involves the mandible , the role of the periodontologist is extremely important . gorham 's disease should be included among the pathologic entities mimicking periodontal disease on radiograph , such as inflammatory disease ( e.g. osteomyelitis ) , endocrine disease ( e.g. hyperparathyroidism ) , intraosseous malignancies or metastases , lymphoma , histiocytosis x , mainly eosinophilic granuloma , infective process ( e.g. tuberculosis and actinomycosis ) and odontogenic tumors [ table 2 ] . the diagnosis is based on anamnestic data ( nonhereditary ) , biochemical data ( absence of nephropathy ) , radiographical data ( progressive monocentric osteolysis without periosteal reaction ) and histological data ( intraosseous angiomatosis with either capillaries or lymph vessels , or both ; eventually fibrosis ) . it is therefore important for oral pathologists to be aware of its existence as a rare case of osteolysis in maxillofacial skeleton . we believe that disease is not as rare as it seems , but rather remains misdiagnosed .
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 .