Pathological changes of the skin usually lead in every case to the fact that the affected persons suffer from psychologically and have to experience a considerable impairment of their quality of life. Nevertheless, the options for treatments are also quite favorable in scleroderma.
What is scleroderma
Scleroderma, properly progressive systemic scleroderma, is a skin disease that is one of the autoimmune diseases. In the processes that take place within the scleroderma, especially the collagen connective tissue, which is so important for the skin, is affected. A so-called sclerotization of the supporting tissue, which is elastic to a certain degree, occurs. For this reason, scleroderma is a collagenosis. This means that scleroderma is classified among the rheumatic systemic diseases of the vascular and connective tissues, which are chronic and characterized by inflammatory processes. Scleroderma is also known by other synonyms such as systemic sclerosis, diffuse or systemic scleroderma. More women than men suffer from scleroderma.
Causes
What the origin of scleroderma is based on has not been clarified up to the present time. Only direct links between progressive systemic scleroderma and overreactions or malfunctions in the body’s immune system are assumed. This fact makes it difficult to find a causal therapy against scleroderma. Intensive scientific research has shown that the cellular components of the connective tissue involved in scleroderma do not function properly, resulting in malformations of the collagenous tissue. In this context, overproduction of collagen occurs in scleroderma, which contributes to hardening and loss of skin tone. Due to the extreme concentration of collagen, fibrosis develops in progressive systemic scleroderma.
Symptoms, complaints, and signs
Scleroderma is usually manifested by episodic circulatory disturbances of the hands and feet. This Raynaud’s phenomenon is accompanied by a striking pallor, as well as pain and/or numbness. If these circulatory disorders persist over a long period of time, the small blood vessels of the fingers and toes suffer permanent damage. Inflammation of the skin occurs, as well as swelling of the fingers, feet, lower legs and toes. Over time, the skin increasingly hardens and also thickens. Due to these changes and poor circulation, it is very pale and has a waxy appearance. The hardening of the skin means that patients can no longer move their joints well. In particularly severe cases of the disease, there is a shrinkage of the subcutaneous fatty tissue. As a result, the fingers appear pointed and narrow. These Madonna fingers are associated with a loss or limitation of fine motor skills. Thus, people with scleroderma at this stage have difficulty tying their shoes or buttoning a shirt. The skin at the tips of the fingers, which is poorly supplied with blood, can die. This causes small painful wounds called rat bite necrosis. Since the skin on the face is also affected by the changes, the patients’ facial expressions change. They can no longer open their mouths properly and have difficulty eating.
Diagnosis and progression
As a consequence of fibrosis manifesting in the skin, solid areas develop in the connective tissue layers in scleroderma. In scleroderma, these contribute to the inability of blood vessels to expand sufficiently, thereby promoting an undersupply of surrounding tissue. In addition, scleroderma causes scars to form in the skin. The course of progressive systemic scleroderma is characterized by acral limiting or diffuse progression. The treatment depends on the skin changes that occur in each case. In the specific diagnosis of scleroderma, physicians have various methods at their disposal. In addition to the classic clinical appearances, it is the examination of skin tissue and a determination of endogenous antibodies as well as the blood sedimentation rate to detect scleroderma.
Complications
The complications of scleroderma are oriented to the type of disease and the involvement of internal organs. Circumscribed scleroderma, for example, is not life-threatening.However, if the disease affects the lungs, pulmonary hypertension may occur, which is associated with severe complications in the further course and may even lead to the death of the patient. Occasionally, the typical edema becomes inflamed and scars or nodules develop. In most cases, a so-called secondary Raynaud’s syndrome also develops, in which the skin areas become pale and the fingertips scar. In the most severe cases, the tissue may even die. Skin tension in the face leads to so-called mimic rigidity, which is associated with premature wrinkling and skin irritation. In some cases, the skin changes permanently and pigmentation disorders develop. In the further stages of the disease, joint inflammation, difficulty swallowing and pulmonary fibrosis occur. All these complaints are associated with further complications. The treatment itself is usually risk-free. Complications can arise, for example, from the prescribed medication, in the course of occupational therapy or during acupuncture. Recurrence of scleroderma after completion of treatment also cannot be ruled out.
When should you see a doctor?
Scleroderma should always be treated by a physician. In this case, without treatment, the quality of life of the affected person is significantly reduced, so treatment should be carried out at the first symptoms and signs of scleroderma. A doctor should be consulted for scleroderma if the affected person suffers from severe disturbances of the blood circulation. As a rule, numbness occurs, so that even ordinary movement is no longer possible without further ado. In many cases, a permanent pallor or severe swelling of the hands may also indicate scleroderma and should be investigated if it persists over a long period of time and does not disappear on its own. In many cases, affected individuals can no longer perform activities with their fingers and hands without discomfort. In the case of scleroderma, a general practitioner or an orthopedist can be consulted in the first instance. Further treatment is then usually carried out by a specialist and depends on the exact manifestations of the symptoms. As a rule, the life expectancy of the affected person is not reduced by scleroderma.
Treatment and therapy
Treatment of progressive systematic scleroderma includes selected procedures that are generally not new. Basically, scleroderma cannot be cured. However, since numerous physical complaints are caused by the progression of scleroderma, treatment is primarily aimed at improving the quality of life. The focus of the entire therapy concepts against scleroderma is the preservation of mobility. For this reason, in addition to special physiotherapy and occupational therapy, so-called physical treatments are also central to the treatment. Since the affected persons suffer strongly, heat-therapeutic applications are to bring with a relief. Of course, these procedures as well as massages and, as an alternative, acupuncture are supported by medicinal agents in the case of scleroderma. Scleroderma not only brings persistent discomfort and intense health limitations. Scleroderma is likewise known to bring about complications. These are presented by the use of immunosuppressants, preparations containing acetylsalicylic acid to promote blood flow, and ACE inhibitors and glucocorticoids.
Prevention
To date, there is no actual prevention against the occurrence of progressive systemic scleroderma. Only extensive professional monitoring and care during scleroderma can reduce worsening of symptoms and potential risks. Prophylaxis in this context includes continuous monitoring of vital functions, regular skin care and skin observation, proper eye care, and avoidance of nicotine and alcohol. Long-term applications, which have already been described in the context of available treatments and are exceedingly useful, also help against deterioration of scleroderma.
Aftercare
As a rule, the measures or options for aftercare in scleroderma are significantly limited or not available to the affected person at all.They are primarily dependent on a rapid and, above all, early diagnosis of this disease so that complications or further spread of the tumor do not occur in the further course of the disease. As a rule, self-healing is not possible with this disease. Treatment depends strongly on the severity of the scleroderma, so that no general course can be predicted. In most cases, however, patients are dependent on the intake of various medications, whereby attention must always be paid to correct dosage and also regular intake. If there are any uncertainties or side effects, a doctor should always be consulted first. During treatment, regular checks and examinations by a doctor are usually very important in order to detect further damage to the internal organs. Most patients also depend on the support and help of their own family during treatment. In some cases, scleroderma also reduces the life expectancy of the affected person.
Here’s what you can do yourself
To avoid triggering a worsening of the skin’s appearance or health, the affected person should discuss the use of cosmetic items such as creams or lotions with the treating physician. There may be ingredients in the skin care products that lead to an increase in symptoms. Therefore, before using cosmetics, it is advisable to check the ingredients used. The symptoms of the disease include physical discomfort, which is perceived as burdensome, especially in everyday life. Physiotherapeutic training is therefore offered in a developed therapy plan. Outside of the treatments, the affected person can also perform learned exercises independently and on his or her own responsibility. This helps and supports the body in improving the overall situation. It also promotes mobility and prevents possible situation of deterioration. Since there is no self-healing in this disease, nor are alternative healing methods sufficient, cooperation with a doctor is especially important. Regular skin care and observation of skin changes is recommended. To promote blood circulation, care should be taken to optimize food intake for blood circulation. In addition, rigid postures should be avoided, as they can impede blood flow. Balancing movements of the limbs should be performed in everyday life. As soon as there is a tingling sensation on the skin, the body position should be changed.
