Intracranial Hemorrhage in a Patient Suffering from Systemic Scleroderma

Scleroderma is a rare connective tissue disease characterized by fi brosis and changes in small blood vessels. Th e cause and pathogenesis are not known, however three possible mechanisms have been proposed: changes in collagen mechanisms, vascular changes, and immunological changes. Th e disease affects various systems, mostly the skin, lungs, gastrointestinal tract, heart, kidneys and musculoskeletal system. In a minority of cases, it may involve the nervous system, mostly due to vasculitic changes. Involvement of the peripheral nervous system manifests with polyneuropathy, whereas central nervous system manifestations include transverse myelitis and hemorrhagic or ischemic stroke. Hemorrhagic stroke can occur as a result of hypertensive crises due to kidney disease and as a hemorrhagic syndrome within immunological thrombocytopenia, when symptomatic epileptic seizures may also occur. In addition, neurological symptoms may be a consequence of calcium deposition in nervous system structures. Our case report indicates the necessity of a multidisciplinary approach in the treatment of this disease and underlines good communication between the physician and the patient as a prerequisite for adequate immunosuppressive and symptomatic therapy.