Treatment of localized and systemic scleroderma

The term scleroderma describes a heterogenous disease characterised by the excess deposition of collagen in skin and/or internal organs. Scleroderma may manifest as a form limited to the skin or as a systemic disease. Circumscribed scleroderma or morphea (limited to the skin) can occur on any area of the integument, however, involvement of the internal organs is extremely rare or completely absent. Although the clinical course of the disease is often benign, widespread lesions and disabling joint contractures may lead to significant morbidity. Systemic scleroderma belongs to the group of "diffuse inflammatory connective tissue diseases" comprising a variety of severe, sometimes life-threatening systemic diseases which often have a chronic, debilitating course. Systemic sclerosis (SSc) is characterized by the involvement of the skin and various internal organs (e.g. kidney, lung, heart). The inflammatory and fibrotic process distroys the normal architecture of the affected organs leading to dysfunction and failure. The severity of the disease process in systemic sclerosis leads to a reduced lifespan, impaired mobility and loss of autonomy.