Gorham–Stout disease or new entity on the basis of vasculopathy

Abstract

Gorham–Stout disease (GSD) is a rare osteolytic bone disease also known as vanishing bone disease. The pathogenesis of GSD is not well understood. Studies showed that lymphatic and blood endothelial cells in addition to macrophages secrete TNFa and IL-6 that stimulate osteoclast formation with osteolysis. Also TNFa secretion inhibits osteoblast differentiation and new bone formation. It is known that cytokines such as TNFa, IL-1 and complement system activation are responsible for inflammation and necrosis in the vessel wall at vasculitis. Both diseases have similar pathogenesis. Here, we presented a case of Gorham’s disease with involvement of bilateral humerus, systemic arterial thrombosis and mesenteric vasculitis.

Keywords: Gorham–Stout disease; Vanishing bone disease; Vasculitis; Thrombosis