Scleroderma is an autoimmune disease. It is an inflammatory rheumatic disease that belongs to the collagenoses. The disease is characterized by progressive hardening of the connective tissue. Initially, the fingertips become discolored only temporarily. Then the skin on the hands, feet and face thickens, becomes hard and brittle. Later, the changes spread to the arms, legs and torso. In the worst case, the connective tissue on internal organs also transforms, with unpleasant to life-threatening consequences.
What are the manifestations of scleroderma?
Connective tissue is found in all human organs and – depending on its composition – performs numerous defensive, nutritional and support functions. In 40 to 220 people per million population, this organ framework increasingly thickens and hardens in single or multiple areas of the body. This leads to a loss of elasticity of the skin – patients feel as if they are in a tight shell.
Even though the disease is relatively rare, for those affected it is often accompanied by severe limitations in quality of life or even life expectancy. It mainly affects middle-aged women, but the disease even occurs in childhood. Scleroderma is classified as a collagenosis, a group of diseases associated with inflammatory connective tissue changes of various types.
Causes of scleroderma
The name is derived from the Greek terms skleros (= hard) and derma (= skin), which already characterizes the clinical picture quite aptly. As a result of an increase in connective tissue, the skin and/or mucous membrane thickens and hardens. The changes progress and can in principle 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). However, the exact cause is unknown.
The extent to which additional genetic factors or disorders of connective tissue formation or vascular regulation play a role is still unclear. In addition, the influence of viral or bacterial antigens, UV light, environmental toxins, sex hormones, medications and certain tumors is being discussed. In the circumscribed form of scleroderma, Borrelia was also long suspected as a trigger, although this has recently been discarded.
