Scleroderma, literally translated as “firm skin”, is a rare inflammatory disease which belongs to the group of connective tissue diseases. Collagen is an important component of connective tissue. Tear strength of ligaments and tendons, flexibility of bones, or pressure resistance of joint cartilage are mainly due to collagen present in the tissue.
In scleroderma connective tissue is hardening, losing its elasticity, and as a consequence losing its functionality. However, connective tissue is not only present in the skin. Blood vessels, bones, cartilage, musculature – all these structures partially consist of connective tissue and thus also of collagen. Scleroderma is not only associated with a great deal of discomfort and complaints, it does not look aesthetic as well and therefore it may influence the patient’s self-confidence negatively.
Types of Scleroderma
A rather localized type of scleroderma, called Circumscribed Scleroderma (CS) or Morphea, is confined to the connective tissue of the skin in a circumscribed, sharply-delimited kind. For the patient it is an annoying and often restrictive disease but it is not life-threatening. Women suffer about three times more often than men and the typical age of onset is between 20 and 50 years of life. The number of people newly diagnosed with scleroderma varies between 2 and 3 to every 100,000 inhabitants annually.
The Progressive Systemic Scleroderma (PSS) also affects the vascular system and inner organs (e.g., lungs, stomach, intestine, heart, kidneys) to different degrees. Depending on the involvement of organs and the progression of the disease severe dysfunctions, physical impairment, and a greatly reduced quality of life can develop. Systemic scleroderma is less common than morphea. In this case the incidence comes to 0,5 to 1,5 per 100,000 inhabitants annually.
Patients with systemic scleroderma often complain about sensitivity to cold during the cold season. It not infrequently happens that their fingers are clearly turning blue as a sign for PSS. In about 60 to 90 percent of the cases this so-called Raynaud’s Phenomenon often precedes systemic scleroderma as initial symptom. During the further course of the illness typically hardening in the area of the fingers occurs, which accompanies with restricted mobility (e.g., aggravated or painful fist closure). In the facial area a pointed nose, narrow lips, and wrinkles around the mouth can develop. In an advanced stage of PSS superficial wounds on fingertips and tiptoes, but also loss of distal phalanx can occur.
Apart from skin hardening different organ systems can also be affected. Often the digestive tract is involved, in particular the esophagus. The complaints could reach from minor to severe swallowing difficulties. Furthermore, the lungs are often affected as well. Lung structure could harden, which later on leads to ventilation problems. People concerned are no longer as capable as prior to disease and are getting out of breath already after minimal physical activity. Heart and kidneys are concerned much rarer.
Therapy of Scleroderma
The therapy of morphea depends on the type of illness. Limited CS does not cause pain and is a purely aesthetic problem. At the beginning it is important to have frequent check-ups instead of a drug therapy, because spontaneous involution can occur. A drug therapy is known and used as well. This way of therapy tries to decelerate the inflammatory process. In particularly complex cases there is a need for a surgical procedure.
Therapy of progressive systemic scleroderma also depends on the degree of severity. In PSS without involvement of any organs there is a focus on sufficient circulation of hardened locations. That is accomplished by medication. In winter it is recommended to wear gloves in order to protect fingers against cold. Smokers shall renounce nicotine during a therapy or reduce the nicotine consumption. In PSS with organ involvement it is important to bring down inflammatory processes in the body systemically with drugs.
Stem Cell Therapy of Scleroderma
A French study has shown that a stem cell therapy with Mesenchymal Stem Cells (MSC) or Stromal Vascular Fraction (SVF) extracted from the patient’s own fat tissue had led to a significant improvement of the general state of health. In the scope of the therapy adult stem cells are extracted from a portion of body fat harvested by liposuction and directly afterwards administered intravenously. In morphea stem cells are injected into the affected tissue areas.