Degos disease, not just a scar: lethal outcome in spite of immunomodulatory therapy.

The article presents a case study of a 4-year-old boy presented an 11-month history of headaches, paresis and subdural hygromas. His physical examination showed scarce papules with an atrophic white centre and rim of erythema located on the abdomen, internal aspect of both ankles and scrotum. He was diagnosed with Degos disease (DD) and treated with infusions of eculizumab and subcutaneous treprostinil and pathomechanisms of endothelial dysfunction, coagulation abnormalities and vasculitis.