Skleroderma

Skleroderma
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Definition

Skleroderma is a group of rare diseases that cause thickening or hardening of the skin. The word scleroderma comes from the Greek, namely "sclero" which means hard, and Latin "derma" or skin. The appearance of this disease varies with each individual. Skleroderma is non-communicable and is not virulent. Although usually this disease only affects the skin, skewloderma can also cause disturbances in blood vessels, internal organs, and cerna channels.

Skleroderma is an autoimmune disease, which is a disease where the immune system attacks the body's own cells. This disease is classified as rare, in the United States there are 75 to 100 thousand people who suffer from this disease, and most of them are suffered by women aged 30-50 years.

 

Cause

Skleroderma occurs due to excessive production and buildup of collagen in the body's tissue. Collagen is a type of protein that forms a tissue to bind to the body, including the skin. Until now, it is not known exactly what causes the scleroderma, but it is estimated that there are roles in several combinations of factors such as disorders in the immune system, genetic factors and triggers from the environment. Research is still underway to find out how the relationship between the slepterma and the immune system is.

 

Risk Factors

Everyone can be exposed to scleroderma, but this condition is much more common in women than men with a ratio of 4:1. This disease most often appears at the age of 25 to 55 years although the scleroderma can be found at all ages, ranging from children to the elderly. There are several factors that are expected to increase the risk of a person experiencing a scleroderma, including:

Genetic factors

Most people do not have relatives or children who also suffer from scleroderma, although there are several cases where scleroderma is found in the family. However, research has found that there is a variety of genes that may increase the risk of a person experiencing scleroderma, although genetic factors alone cannot cause the emergence of a disease.

Environmental factors 

Research shows that in some people, the symptoms of scleroderma may be triggered by exposure to certain viruses or drugs. Repeated exposure to chemicals that are harmful, such as at factory workplaces, can increase the risk of cleroderma. However, environmental factors do not always play a role in everyone.

Immune system disorders 

Skleroderma is believed to be an autoimmune disease, in which immune cells attack themselves, in this case, a connective tissue in a healthy body. Sclerodermic sufferers may also have other autoimmune diseases such as batromatic or lupus.

 

Symptoms

Skleroderma is a chronic disease in the body's tissues, and the appearance of the symptoms can be very different from person to person, it can be mild to life-threatening. Symptoms can be seen clearly if the sufferer's skin is affected, but it can also not be seen clearly if the internal organs are affected. Symptoms can appear in the eyes, mouth, hands, joints, kidneys, heart, lungs, brain, or digestive tract.

Symptoms that can be seen in scleroderma depending on the organs or tissues affected, among others:

  • The thickening and swelling of the skin
  • Swelling on the finger and clearly visible when waking up in the morning, the symptoms that usually appear at the beginning of the scleroderma
  • Strong taste in the face
  • It is difficult to move the lips and mouth
  • Sjoorgren syndrome is an autoimmune disease experienced by 20% of scleroderma sufferers, where there is a drought in the mouth and eyes, difficulty speaking and swallowing, as well as a decrease in taste
  • Phenomenon Raynaud, this occurred due to the installment of the diameter of small and abnormal blood vessels on the fingers and feet
    • Fingers can turn pale, bluish, or red
    • Feeling numb or pain
  • Sendi teraba hangat atau sakit
  • Weakness and muscle aches
  • Heartburn or burning in the chest
  • Diarrhea
  • Difficult to swallow
  • Constipation
  • Shortness of breath
  • Cough persistent
  • High blood pressure
  • Headache
  • Visibility disorders
  • Chest pain, etc.

 

Diagnostic

Skleroderma can be difficult to diagnose because it can cause varied symptoms and hit different parts of the body. After conducting a physical examination, if the doctor feels that the signs and symptoms you feel refer to conditions that require further examination, the doctor may suggest that you do some supporting examinations. 

Doctors will usually do blood tests to find autoantibodies in the blood. Autoantibodies are proteins produced by the immune system and attack healthy body tissues themselves. This diagnosis process usually involves other specialists such as autoimmune disease specialists or skin specialists. Consulting with other specialists depends on which organs are affected.

Other supporting checks carried out can also help:

  • Confirming diagnosis
  • Finding out if there is a risk of complications
  • Observing inflammation and autoantibody levels in the body
  • Seeing the journey of disease and the level of damage that occurs in the body's organs

 

Laksana Procedure

Currently, there is no definite therapy that can cure scleroderma. Although there are many therapies available to treat the symptoms that arise, no drugs are proven to stop or restore the body's original condition. Currently, the treatment provided is in accordance with the symptoms that arise in the sufferer, to prevent complications, and improve the quality of life of the sufferer. In some patients with mild symptoms, there are some people who do not require therapy, or therapy is stopped when the scleroderma is no longer active. Some of the therapies given are also intended to reduce the activity of the immune system.

Drug choices can vary depending on the body part affected. The drug used is usually a drug for:

 

Laksana Procedure

While there is no treatment to cure scleroderma, therapy can help reduce symptoms, slow disease progression, and improve quality of life.

Drug choices can vary depending on the body part affected. The drug used is usually a drug for:

  • Widening the blood vessels to improve the symptoms of the phenomenon Raynaud
  • Press the immune system, to reduce the progression of scleroderma such as thickening the skin or lung damage
  • Reducing digestive symptoms, these drugs include drugs to reduce stomach acid, as well as medicines and antibiotics to help digestion reduce symptoms of begah, diarrhea, and constipation
  • Preventing infections that can worsen lung conditions that are already exposed to scleroderma
  • Pain loss

In addition to drugs, physical therapy or rehabilitation can help to increase strength and movement so that patients are able to carry out their activities as normally as possible. In cases that are severe and do not respond to the therapy that has been given, there is an option for punca cell transplantation. In severe lung or kidney damage, organ transplantation can be taken into consideration.

 

Complications

Skleroderma can cause complications, ranging from mild to severe. Complications can occur in:

1. Fingers

In scleroderma, there can be a Raynaud phenomenon that is so severe that it inhibits blood flow and causes permanent tissue damage to the tip of the finger. This causes skin injury. In some cases, the tip tissue can experience death.

2. Lungs

Lung damage can interfere with the respiratory process and reduce the ability to carry out activities. In addition, lung hypertension can also occur.

3. Kidney

Serious complications due to scleroderma can occur in the kidneys characterized by sudden increase in blood pressure and rapid kidney failure. Therapy is immediately essential to maintain kidney function.

4. Heart

The thickening of the heart tissue can cause heart rhythm disorders and heart failure. Scleroderma can also cause inflammation in alcoons that surround the heart.

5. Gigi Gingi

Heavy facial skin thickening can cause the mouth to become smaller and narrower so that it is difficult to clean the teeth. In addition, scleroderma sufferers often experience problems with saliva production so that the risk of dental caries increases.

6. Cerna channel

Cerna channel problems related to cleroderma can cause symptoms of a burning chest and difficulty swallowing, accompanied by stomach cramps, rebuttal, constipation, or diarrhea. Some people can also experience nutritional absorption problems due to abnormal growth of bacteria in the intestines.

7. Sendi

The skin above the joints can thicken and interfere with joint movement, especially in the hands.

 

Prevention

Because the exact cause is not yet known, there is no way to prevent scleroderma. Scleroderma disease can be triggered by a combination of genetic factors, environmental factors, and disorders in the immune system. If you have family members who experience scleroderma, there is nothing wrong with doing genetic screening. Avoid exposure to harmful chemicals and use personal protective equipment if you work in a vulnerable factory or place.

 

When Do You Have To Go To A Doctor?

If you experience symptoms of scleroderma, then you should consult a doctor to get early and proper treatment.

 

Want to know information about other diseases? Check here, yes!

 

 

Writer : dr Tea Karina Sudharso
Editor :
  • dr Hanifa Rahma
Last Updated : Senin, 30 Mei 2022 | 07:57

Scleroderma - Diagnosis and treatment - Mayo Clinic. Mayoclinic.org. (2022). Retrieved 19 May 2022, from https://www.mayoclinic.org/diseases-conditions/scleroderma/diagnosis-treatment/drc-20351957.

Scleroderma. Rheumatology.org. (2022). Retrieved 19 May 2022, from https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma.

Understanding Scleroderma - National Scleroderma Foundation. National Scleroderma Foundation. (2022). Retrieved 19 May 2022, from https://scleroderma.org/understanding-scleroderma/.