Scleroderma is a disease that causes skin problems in particular, making the skin look ugly and hard. Skin isn’t the only thing that scleroderma affects. You can find more information below.
What is scleroderma?
Scleroderma is a connective tissue disease characterized by abnormal production of collagen, a fiber-like protein. Collagen is a necessary component of connective tissue, but too much collagen overwhelms tissues, causing the tissues to harden and tighten, causing inflammation, damage, and dysfunction in the skin, bones, muscles, and other body organs. Scleroderma is thought to result from an abnormal immune system response.
Does scleroderma kill?
Although scleroderma is a chronic disease that does not go away, it will not kill you. Getting treatment can put you at ease, help control the disease, and prevent it from getting worse.
Types of scleroderma
1- Localize scleroderma
Localized scleroderma, the more common form of the disease, affects only a few places and only the skin of the person concerned. It usually appears as waxy patches or lines on the skin. Although localized scleroderma sometimes goes away on its own, it is important to see a doctor and get treatment.
2- Systemic scleroderma
The systemic form of scleroderma affects many parts of the body. Not only does it affect the skin, it can also affect many internal organs, such as the kidney, which hinders digestive and respiratory functions. Systemic scleroderma can sometimes become a serious and life-threatening condition.
Causes of scleroderma
Although scleroderma is thought to be an autoimmune disease, its exact cause is not yet known. As with almost any disease, there may be a genetic predisposition to develop scleroderma. Infections caused by viruses, bacteria, tumors, certain hormones and certain drugs are considered possible triggers of scleroderma .
What are the symptoms of scleroderma?
1- Raynaud’s syndrome
We all know our body’s normal response to cold. Our body reduces heat loss by increasing blood flow from superficial veins to deeper body veins to protect against cooling.
In Raynaud’s syndrome, abnormal regulation of the autonomic nervous system causes excessive vasoconstriction of blood vessels and seizure-like cramps. These shrink so much in the cold that they cut off blood flow for a few minutes before being released again. Fingers or toes turn white, then purple, and finally red. White indicates interrupted blood flow, purple indicates a lack of oxygen in the tissue, and increased blood flow towards the end is red.
A Raynaud’s attack is associated with a tingling sensation and sometimes severe pain . Other areas exposed to cold are rarely affected, such as the nose or ears. Raynaud’s phenomenon is not a typical but clear precursor of scleroderma.
2- Cilt sclerosis
Collagen fibers of connective tissue proliferate and harden all over the body of scleroderma patients. Those affected describe a feeling of tension, the whole body contracts and slows down movements. Skin sclerosis initially thickens and hardens the skin of the face, skin, and fingers. It feels tight and has a waxy sheen. At the same time, the lubricity of the skin is impaired. The affected area is no longer so easy to move.
Tight, slippery skin impedes movement of the small joints in the fingers and toes. Around the mouth opening, it can restrict food intake and dental care. There may be visible red skin veins, especially in the face and décolleté area. Some distortions can also be seen in the nail area.
As the disease progresses, the skin often becomes thinner and the underlying fatty tissue also decreases. It can narrow between the toes and resemble a claw in a vulgar expression. Finally, facial expressions may freeze and the face may look like a mask. Those affected then have trouble closing their eyelids or opening their mouths.
3- Joints and muscles
Many people with scleroderma have problems with the musculoskeletal system. These problems are mostly in the fingers and wrists, but also in the elbows or hip joints. Joints can become stiff, inflamed, and cause severe pain. Hard tendons and nerve disorders ( carpal tunnel syndrome ) are also seen in the wrist.
4- Wounds on the skin (ulcers)
About half of all scleroderma patients suffer from sores that are very painful and only slowly heal. It occurs during the disease, especially on the skin of the fingers, toes, or in the elbow area. The main cause of these sores is poor blood circulation in the tissue, as a result of which the blood vessels in the scleroderma are damaged, narrowed and destroyed.
In addition, hardened or deformed joints or lime deposits in the subcutaneous tissue can lead to non-healing skin sores.
5- My nervous way
- Esophagus :
The esophagus is the most commonly affected area. Hardening of the connective tissue can restrict the mobility of the esophagus, thereby slowing the transport of food to the stomach.
- Stomach :
The mobility of the stomach and thus its functioning may be impaired. Then the food stays in the stomach much longer, so the feeling of fullness happens faster or very soon. If the gastric inlet is affected, gastric juice may enter the esophagus and heartburn may occur.
- Intestine : Scleroderma can affect the gut, especially the small intestine. On the one hand, the small muscles in the intestinal wall suffer from an insufficient supply due to circulatory disorders. On the other hand, the growth of connective tissue can thicken and harden the intestinal wall. Both types of damage slow intestinal transit, which can lead to bloating and irregular stools.
6- Heart and lungs
If scleroderma spreads to the connective tissue in the heart muscle, it can lead to heart muscle disorders or heart arrhythmias. If it affects the blood vessels of the lungs or the lung tissue itself, there is difficulty breathing during exertion and a dry cough. The thicker and harder the connective tissue of the lungs becomes, the more gas exchange (oxygen-carbon dioxide) is affected.
As a result, the heart has to pump more blood through the circulation. This additional performance is a heavy burden on the heart. Lung diseases are the leading cause of death in people with severe scleroderma. Heart failure is also a common cause of death.
If circulatory disorders affect the fine vessels of the kidneys, this can seriously impair kidney function.
- Greatly increased blood pressure
- visual disturbances
- Shortness of breath
- leg swelling
- Foam in the urine (sign of increased urinary protein excretion)
Kidney problems can develop very quickly and require immediate treatment.
8- Depression and sexual dysfunction
Scleroderma patients also suffer from psychological problems. One in two affected people show mild to severe symptoms of depression. Depressed mood has a negative effect on all other complaints.
Sexual disorders also occur. Circulatory disorders cause erectile dysfunction (impotence) in four out of five male patients . Vaginal dryness in women can also affect sex life.
Like many chronic diseases, scleroderma can lead to energy deficiencies, chronic fatigue and exhaustion.
How is schloroderma diagnosed?
Scleroderma is sometimes difficult to identify. Different internal organs can be affected to varying degrees. Mixed forms associated with other rheumatic diseases such as rheumatoid arthritis , lupus (butterfly) disease, or Sjögren’s syndrome are also possible. Therefore, the diagnosis should be made by an experienced specialist. The doctor will ask the person concerned about their complaints and monitor changes using a standard procedure, especially a thorough physical examination of the skin .
Blood tests can usually (but not all patients) be examined in the laboratory to detect certain antibodies. An important examination method is capillary microscopy. Capillary microscopy determines blood flow and the condition of the smallest blood vessels in the nail bed. At this point, the best blood vessels lie just below the surface and are easy to count, measure, and evaluate.
In addition, the chest and possibly the digestive organs, heart and lungs are examined to determine the overall severity of the disease and the specific organ condition. Common diagnostic procedures include lung function tests and computed tomography .
How is schloroderma treated?
Scleroderma is not easy to treat. Those affected must take personal responsibility and be willing to actively help themselves, because medicine alone is not enough.
1- Drug therapy
Drug therapy is based on the individual characteristics of scleroderma. Vascular dilation medication is recommended for Raynaud’s syndrome . In the case of skin wounds, other vascular protective drugs can be administered. However, the main goal of drug therapy is to inhibit the spread of connective tissue proliferation. Depending on the organ affected, you can use different drugs. For example, very effective drugs lower common pulmonary hypertension , and people with heartburn can take acid blockers to prevent inflammation of the esophagus .
Treatment of scleroderma should be tailored to the individual patient. Doctors from different disciplines such as rheumatology, cardiology and pneumology work together. If skin sclerosis causes severe body itching, antihistamines may provide relief. If the skin is burning, some ointments can help calm the skin nerves.
Remember, your doctor will decide which medicine to take.
Various non-drug treatments
Numerous treatments help against the pain, swelling, and various physical limitations of scleroderma, such as:
- Manual lymphatic drainage
- Heat treatments such as hyperthermia
- Various exercises
- Mobilization of joints
- muscle building training
- endurance training
- respiratory therapy
Large muscle groups in the legs and trunk are important sources of energy. Shaping them with strengthening exercises and sports activities prevents fatigue and exhaustion. Endurance training is recommended, especially in the case of frequent pulmonary hypertension . In addition, endurance training promotes blood circulation and thus the delivery of oxygen to the tissue, often improving mood and mental well-being.
What is good for scleroderma?
- Avoid smoking completely
- Protect hands from cold, wetness and cuts
- Make paraffin baths for hands
- Apply skin care with oily creams
- Take care of your oral and dental health
- for lots of water
- Learn relaxation techniques
Living with scleroderma
The best thing you can do is work with your doctor to manage symptoms and prevent complications. However, there are still things you can do on your own that can help.
If you have skin problems , try to keep your skin soft and moist. Use sunscreen outside and a steamer at home. Avoid harsh skin products, hot showers and baths. If you have heartburn or other digestive problems , try to eat small and frequent meals. Do not lie down immediately after eating. Avoid spicy foods, alcohol, caffeine, and other things that trigger heartburn.
If you have Raynaud’s disease, you can:
- avoiding smoking
- Wearing warm clothing, including socks and gloves if necessary
- do regular exercise
- avoiding things that cause increased stress or anxiety
You should talk to your doctor about other methods to help improve blood flow. Doctors can prescribe medications to treat skin blemishes, sores, and lesions.