Köhlmeier-Degos disease: Does the benign form really exist?

A 46-year-old woman was referred to us for evaluation of asymptomatic erythematous papules with central umbilication and atrophy in trunk and extremities. Histologic features were compatible with Degos disease. She had no systemic manifestations and the laboratory and the radiologic findings were no significant. 6 years after the diagnosis the patient complained of headache and weakness. Initial evaluation revealed a subdural hygroma on CT. Then she developed a hemiparesis, and MRI showed a subacute ischemia in the territory supplied by the middle cerebral artery.