Progressive Gorham Disease of the Forearm

Gorham's-Stout disease is a rare but potentially debilitating disease consisting of massive bone osteolysis and bone resorption associated with vascular proliferation and increased osteoclastic activity. Although it can present in a wide variety of forms, it typically involves bones formed by intramembranous ossification such as the skull, pelvis, and scapula. It can occur spontaneously or after trauma. Most cases are monofocal and resolved spontaneously, although there are reports of multifocal and rapidly progressing disease. It typically presents as disuse muscle atrophy or pathologic fracture during the second through fourth decades of life, yet it has also been reported in childhood and in the elderly. The etiology of Gorham's disease remains to be fully elucidated. Gorham attributed the origin of the disease to uncontrolled proliferation of small vessels eating away bone tissue. Other authors attribute the cause of the disease to increased osteoclastic activity mediated by elevated cytokine levels and increased osteoclastic differentiation. Treatment is not established and focuses at stopping osteoclastic activity and angiogenic proliferation. Radiation therapy, chemotherapy, bone grafting, and antiresorptives medications have all been used with different degrees of success. In an effort to further characterize this elusive disease, we report on an unusual presentation of a patient with Gorham's disease of the radius spreading to the ulna and then the proximal humerus with a 13-year follow-up. To our knowledge this is the first report in the literature of a saltatory type of Gorham's disease spreading from bone to bone across a joint.