Scleroderma

The name comes from the Greek words skleros, meaning “hard,” and derma, meaning “skin”—literally, “hard skin.”

It is a rare autoimmune disease that affects the connective tissue (connective tissue is found throughout the body). Connective tissue links different cells together and, among other things, provides strength and support.

In autoimmune diseases, the patient’s immune system mistakenly attacks the body’s own tissues.

In Scleroderma, the skin and blood vessels are affected. In addition, internal organs such as the lungs, heart, kidneys, and gastrointestinal tract (particularly the esophagus) may also be affected to varying degrees.

 

The prevalence of Scleroderma is approximately 0.001%.

The condition is more common in women than in men.

Scleroderma is extremely rare in children.

The exact cause of Scleroderma is unknown. For reasons that are still not fully understood, the production of connective tissue becomes disrupted.

In Scleroderma, more collagen is produced than elastin, causing the connective tissue to become thicker and less flexible.

The hereditary component is still not fully understood. Family members may experience rheumatic conditions without necessarily developing Systemic Sclerosis.

Scleroderma is not contagious!

 

This form is characterized by hardening of the connective tissue in the skin and underlying tissues, but it does not affect the internal organs.

 

In this form, the hardening of connective tissue can affect both the skin and the internal organs. Patients with this form are divided into two main groups.

 

This classification is based on the pattern of skin involvement and is important because diffuse and limited Systemic Sclerosis differ in disease progression, organ involvement, and the rate at which symptoms typically develop.

 

This is a form in which not only the skin of the hands/feet, forearms/lower legs and face are affected, but also larger areas of the skin such as entire limbs and the trunk.

In addition, organs such as the heart, kidneys, lungs and gastrointestinal tract are also affected.

The same symptoms also occur in the “limited cutaneous form”.

This form is usually limited to the Raynaud phenomenon only (see below).

 

Limited cutaneous form:

 

In this form, the skin involvement usually remains limited to the hands/feet, forearms/lower legs and face. Organ involvement occurs more slowly than in the diffuse form.

 

Some patients have CREST syndrome. CREST stands for:

C: Calcinosis, calcium deposits.

R: Raynaud phenomenon, discoloration of the fingers due to cold.

E: (o)Esophagus, esophageal involvement.

S: Sclerodactyly, drying and hardening of the fingers.

T: Telangiectasias, small red spots on the skin.

 

These elements can each occur separately or in varying degrees and severity.

 

This form is extremely rare and involves organ involvement without skin changes. (sine = without)

The symptoms caused by Scleroderma vary from person to person. Scleroderma is a very complex condition and, as a result, it can sometimes take weeks, months or even years before a doctor can recognize the condition and finally make the diagnosis!

In the majority of patients, Scleroderma begins with the Raynaud phenomenon.

A smaller group, however, initially experiences more problems with fatigue, hair loss, and pain and stiffness of the muscles and joints.

Swelling and fluid retention can occur in the hands/arms/feet/legs and/or face. As a result, the skin may become shiny and appear tight. The face may become expressionless.

As already mentioned, in a large group (95% of Scleroderma patients), the condition begins with Raynaud phenomenon…

The Raynaud phenomenon can be characterized by the sudden appearance of discoloration of the fingers and/or toes when exposed to cold or during emotional stress. First, a white discoloration occurs, which is caused by the contraction of the supplying blood vessels, preventing blood from flowing properly to the skin. After the white phase, a purple, dark blue phase follows because the blood present in the blood vessels does not circulate properly. Patients experience both phases as painful, and the tingling sensations are comparable to having “sleeping” fingers or chilblains. The discolored body parts become noticeably red after warming due to increased blood flow. Under unfavorable circumstances, the blood vessels remain blocked for a longer period of time and sores can develop on the fingers or toes. Blood vessel inflammation can also occur. Usually, the phenomenon is limited to the hands and/or feet, but other body parts can also be affected, such as the ears and the tip of the nose.

Internally, spasms of the blood vessels can also occur, causing, for example, the heart muscle to temporarily receive less blood, which can result in chest pain. The lungs can also be affected; exposure to cold air can cause shortness of breath. The kidneys can also be affected, which may influence blood pressure.

Exposure to cold should be avoided, and a warm, especially non-humid environment should be sought. When avoiding cold, we should not only think about wearing gloves, but also wearing a hat! Obviously, it is best not to smoke, as this causes the blood vessels to constrict even more. Doctors can prescribe medication that widens the blood vessels; however, one should be aware of possible side effects.

Emotions are of course not so easy to control, but you can try to avoid stressful situations as much as possible… In today’s fast-paced society, this is not always easy.

 

 

Blood tests, urine tests, capillaroscopy, radiography, CT scan, electrocardiogram, echocardiography, lung function tests, 6-minute walking test and esophageal manometry

 

It is extremely important to discuss your desire to have children with your treating physician!

Your doctor will provide appropriate guidance and take care of the health of both mother and child. In many cases, Scleroderma only develops later in life, meaning that the pregnancy has already taken place.

 

Doctors and healthcare teams are mainly concerned with monitoring the vital organs and the skin score, without paying much attention to this important yet very sensitive topic. It is not really a life-threatening problem, but Scleroderma can contribute to having no or less sexual contact. Physical consequences of the condition may be the cause, as well as psychological or social consequences and the use of medication. Physical complaints can result in a reduced desire for sex… Men often experience erectile dysfunction. This disorder occurs because the erectile tissue in the penis does not receive enough blood. In some cases, the skin of the penis becomes hardened, causing changes in shape. As a result, sexual intercourse may become difficult or impossible. Achieving orgasm can also become more difficult. The advice we give is to discuss this with your treating physician!

You can then be referred to a urologist or sexologist for further examination and possible treatment.
In women, the vagina may become drier, which can cause pain during intercourse.

Sometimes the use of a lubricant can reduce these symptoms. Consult a doctor in time and discuss the problem!

In both men and women, hardening of the skin and limitations in movement can result in sexual intercourse becoming more difficult or impossible.

 

In addition to physical symptoms, feelings such as fear and worry can cause you to have less desire for sex. You may find your body less attractive, or you may be tired and in pain—in short, there may be enough reasons to avoid sexual contact or postpone it until a “better” moment. Sometimes feelings of guilt or the feeling of not wanting to burden the other person can strongly affect the relationship. The most important thing is that you talk honestly with your partner about this sensitive and intimate issue. Exploring together what is possible remains essential! Finding other ways of intimacy is a challenge… Give everyone involved time to adjust to the new situation…

 

The condition does not only have an impact on the physical level, but also greatly affects social and psychological well-being.

The extreme fatigue and “strange” pains are sometimes difficult to accept. Everyone has their own way of giving this a place and processing it.

All kinds of emotions can arise, such as fear, sadness, helplessness and anger.

Give yourself time to find a balance in your daily life…

Your surroundings will also need to adjust to the new situation. Support from family and friends becomes very valuable, and accept this support with both hands.

Seek help if necessary from your general practitioner, your treating physician or healthcare team…

LEARN to ‘cope with your Sclero’…

 

Staying positive is not always easy, but it is very important!!

To date, there are still no medicines that can cure Scleroderma.

Treatment differs from patient to patient. Medicines are used, with the choice of medication depending on the severity, nature and especially the extent of the involvement.

Fine-tuning the treatment to suit the individual patient requires considerable experience from the doctor. Good cooperation between a multidisciplinary team and the patient is essential!

The butterfly is an animal that, just like people with scleroderma, needs a lot of rest, but repeatedly finds the courage to continue flying…

The sunflower always turns towards the sun… people with Scleroderma love warmth and feel considerably better because of it…