Scleroderma is an autoimmune disease. It is an inflammatory rheumatic disease that is one of the collagenoses. The disease is characterized by an increasing hardening of the connective tissue. At first, the fingertips change only temporarily. Then the skin thickens on the hands, feet and face, becomes hard and brittle. Later, the changes also affect the arms, legs and torso. In the worst case, the connective tissue also transforms to internal organs, with unpleasant to life-threatening consequences.
How is Scleroderma expressed?
Connective tissue occurs in all human organs and fulfills - depending on the composition - numerous defense, nutritional and supporting functions. With 40 to 220 people per million inhabitants thickened and hardened this organ structure increasingly at one or more points of the body. This leads to loss of elasticity of the skin - the patients feel like in a narrow tank.
Even if the disease is relatively rare: For those affected it is often associated with severe limitations in quality of life or even life expectancy. It affects mainly middle-aged women, but even in childhood, the disease occurs. Scleroderma is attributed to collagenosis, a group of diseases associated with inflammatory connective tissue changes of various kinds.
Causes of scleroderma
The name derives from the Greek terms scleros (= hard) and derma (= skin), which already quite aptly characterizes the clinical picture. As a result of a connective tissue proliferation, skin and / or mucous membranes thicken and harden. The changes continue and, in principle, can affect all parts of the body.
The trigger of scleroderma is a dysregulation of the immune system in the sense of a defense reaction of the body against its own tissue (autoimmune disease). Their exact cause is unknown, however.
The extent to which additional genetic factors or disorders of the formation of connective tissue or vascular regulation also play a role remains unclear. In addition, the influence of viral or bacterial antigens, UV light, environmental toxins, sex hormones, drugs and certain tumors are discussed. In the circumscribed form of scleroderma Borrelia from triggers have long been suspected, and this has recently been discarded.