Gorham's disease
Gorham's disease Causes
The exact cause of the disease is unknown, although several hypothesis about it have been made since the first recorded case in 1954.
Gorham's disease Definition
Gorham's disease, also known as Massive Osteolysis, is a rare congenital bone disorder characterized by the spread of vascular channels that are destructive to the bone matrix. Osteolysis, or vanishing of the bones, occurs as well as angiomatosis.
Gorham's disease Diagnosis
The disease is usually discovered when one has abnormal pain in fractures or bones. Diagnosis is done through x-ray, scanner, biopsie, lymphography, and MRI.
Gorham's disease Symptoms and Signs
The symptoms of Gorham's disease vary from case to case. Typically they include progressive weakness and musculoskeletal pain. In some cases, bone loss is not replaced by new bones. Instead, fibrous tissue may grow in its place, although this leads to a fracture which results in faster progression of the disease. In its early stages, radiographic detection will show decreased bone mass. Gorham's disease may also present itself through acute or chronic pain. Bone deformity occurs in children. Severe cases are characterized by a pleural effusion, or when fluid accumulates in the space the chest cavity and the membrane around the lungs. The bones usually affected include shoulder, arm, hand, part of the pelvis, jaw, spine, skull, spleen, hips, lungs, and femur.
Gorham's disease Treatment
Treatment focuses on hindering the disease progression through therapeutic measures. These include ALPHA-Interferon, Radiation Therapy, and bisphsphonates.