Collagen XVIII and fibronectin involvement in bullous scleroderma.

Background: Endostatin, an anti-angiogenic C-terminal fragment of collagen XVIII, has been recently reported to play a role in scleroderma pathogenesis, but collagen XVIII immunohistochemistry in scleroderma skin has still not been performed. Bullous scleroderma, a rare form of scleroderma, may have altered angiogenic and lymphangiogenic characteristics.

Objective: Our aim is to report a rare case of bullous scleroderma, studying the presence of fibronectin and collagens type I, III and XVIII in sclerodermic skin.