What is Scleroderma?

SCLERODERMA
(Sclero: hard, derma: skin)

Scleroderma (Systemic Sclerosis) is a chronic connective tissue disease, commonly classified as one of the autoimmune rheumatic diseases. Autoimmune is a state where the immune system is overly unnatural, so the immune that is supposed to protect itself against bacteria, viruses, and other foreign attacks, actually attacks and causes damage to the healthy organs in the body.

Scleroderma is not a contagious disease, it does not cause infection, is not cancerous or malignant, and is usually not due to heredity.

ANYONE WHO CAN GET SCLERODERMA?

Statistically, women who suffer from Scleroderma more than men, with a ratio of 9: 1. Scleroderma can be found in every age group, from infant to parent, but most often at the age of 25-55 years.

Factors of gender, race and ethnic background, can affect the risk of scleroderma, but the cause is less clear. Although Scleroderma is not inherited / derived directly, some scientists feel a slight tendency to be exposed to families with a history of rheumatic disease.

CAUSES OF SCLERODERMA

The exact cause of Scleroderma is still unknown, medical scientists and researchers in various fields are working hard to find it. However, there are three things found in Scleroderma, namely:

1. Autoimmune process

2. Overproduction of collagen

3. Damage to blood vessels

Collagen is the main protein of the connective tissue of the body, a network that binds the cells together. Collagen is found in the skin, joints, tendons and parts of the internal organs. Collagen consists of small fibers, interwoven like threads to form a piece of cloth. When the production of collagen is excessive, there is thickening and hardening of the affected area which will ultimately interfere with the normal functioning of these body parts.
There are several theories about how collagen production becomes redundant:

· Autoimmune theory shows that the immune system plays an important role. Usually, the immune system produces chemical signals in the blood called cytokines, which coordinate the body’s defenses against bacteria, viruses and other foreign attacks. In addition, cytokines help repair the wound by stimulating the production of collagen to cover the scar. There are several theories in which the immune system is overly abnormal, causing excessive amounts of cytokines to be produced. These cytokines cause damage to healthy body tissues and can also stimulate the excess production of collagen.

· Vascular theory is related to blood vessels. Damage to blood vessels, especially small ones, is characteristic of Scleroderma. Damage to blood vessels causes blood vessels to contract, become stiff and overreact to cold or stress. These reactions can cause further damage to the blood vessels themselves and the organs of the body, which are supplied by these blood vessels. There is also the possibility of a link between the overproduction of collagen and damaged blood vessels. Damaged blood vessels may allow increased leakage of cytokines into adjacent tissues that can ultimately stimulate the production of excessive collagen.

TYPES OF SCLERODERMA

There are two classifications of Scleroderma, namely Localized Scleroderma (Morphea) and Scleroderma Systemic (Limited and Diffuse). Scleroderma can appear with various symptoms based on the division.

· Localized Scleroderma (Morphea)

In Scleroderma this type of skin body thickened, looks shiny, slippery like a candle and reddish or brown. These changes are usually found only in some parts of the body, and rarely spread elsewhere. These thickening may expand or decrease, and often disappear spontaneously. Morphea typically appears at a vulnerable age between 20-50 years, but it can also affect children. People with Morphea do not have Raynaud’s symptoms and very rare interbals are affected. People with Morphea rarely become Systemic Scleroderma.

· Linear Morphea

Linear Morphea is a form of Local Scleroderma whose symptoms start with the skin, the skin is like having candles on the arms, legs or on the forehead. Sometimes shaped like a long crease on the head or neck, referred to as “en coup de saber” because of its resemblance to an incision or a wound by a sword. Linear scleroderma tends to involve deeper layers of the skin and sometimes restrict movement of joints located under the affected skin. Linear scleroderma usually develops in childhood. In children, and can affect the growth of affected limbs.

· Systemic Sclerosis (Systemic Sclerosis)

Changes that occur in Systemic Scleroderma can affect connective tissue in all parts of the body. Systemic Sclerosis can invade the skin, blood vessels, muscles, joints, esophagus, digestive tract (stomach and intestines), lungs, kidneys, heart and other internal organs. The affected tissue becomes hard and fibrous, causing the functioning of the organs to decrease. There are two main patterns known to cause, limit or increase the severity of the disease. Based on the area of ​​affected skin, the disease is divided into two groups. In general, the affected skin on Scleroderma begins on the fingers and spreads to the arms. Very common skin thickening of the face and feet.

1. Scleroderma Limited (Limited Scleroderma)

Limited Scleroderma usually causes Raynaud’s Phenomenon and Hardening of the skin on the hands. Changes in facial skin, skin on forearms and lower limbs.
Limited Scleroderma. affect other internal organs, such as problems with the esophagus, but it is important to realize that internal organ involvement is usually very mild and likely to occur only after years of illness. Prospect Limited Scleroderma. generally very good. About 70% of people with Systemic Scleroderma have Limited Scleroderma, while the remaining 30% are more severe with Diffuse types.

CREST is another name used to describe a subgroup of people with Scleroderma. CREST is an abbreviation for the clinical combination of Calcinosis, Raynaud’s phenomenon, problems with the esophagus, Sclerodactyly (stiff fingers) and Telangiectasia (small red blood vessels dilated on the skin or face). Most CREST patients have Limited Scleroderma.

2. Scleroderma Diffuse (Diffuse Scleroderma)

Diffuse Scleroderma affects the skin not only on the hands and arms, but also the skin of the whole body. Diffuse Scleroderma has the potential to affect other organs and tissues. This type of Scleroderma often requires more intensive treatment and some patients because this type has a serious disorder.

Diffuse Scleroderma generally has early symptoms fairly quickly, skin thickening occurs within months. However, skin thickening may return after a few years with minimal organ damage in the long run.

HOW IS SERIOUS SCLERODERMA?

Every chronic illness is a serious illness. The symptoms of Scleroderma vary greatly among individuals. Scleroderma effects can range from very mild to life-threatening. The severity of the disease depends on which part of the body is affected and the extent to which it is affected. In some cases, Scleroderma attacks the skin within a few years, then spreads to internal organs. A prompt, appropriate, and appropriate diagnosis and treatment by a proper specialist will minimize the symptoms of Scleroderma and reduce irreversible/incurable damage.

DIAGNOSIS OF SCLERODERMA

Diagnosing scleroderma can be very difficult, especially in the early stages. Many of the symptoms are common, or may overlap with other autoimmune diseases (overlaps), such as overlap with Rheumatoid Arthritis (RA), Sjogren Syndrome (SS), Psoriasis, or Lupus (SLE).
Scleroderma is seen from the more visible symptoms of the eye. No single test can prove its existence. Diagnosis is usually made by your doctor through the following combinations: medical history, including past and present symptoms, a thorough physical examination, and the results of various laboratory tests and other studies. In making the diagnosis, it is important to not only confirm the presence of Scleroderma, but also to determine the extent and severity, especially with regard to internal organ involvement.
Limited and Diffuse Scleroderma can sometimes be distinguished by the presence of different antibodies, called Anti-nuclear Antibody (ANA) in the blood. For example, anti-SCL-70 is more commonly associated with Diffuse Scleroderma, while anti-centromere usually occurs in Limited Scleroderma.