Scleroderma

Definition of scleroderma

Scleroderma is a group of rare diseases that include hardening and tightening of the skin and connective tissue (fibers that hold the skeleton and tampons to the body). In scleroderma, the immune system has a disorder that causes excessive collagen production by the body.

In some individuals, scleroderma only affects the skin (localized scleroderma), but there are many cases where scleroderma also affects other parts of the body (systemic scleroderma) such as blood vessels, muscles, internal organs and digestive system. Local scleroderma is then divided into morphea, general morphea, saber stroke (sword incision), and linear. Systemic sclerosis can affect many systems or parts of the body. This type of scleroderma can also be divided into 2, namely limited scleroderma and diffuse (scattered) scleroderma. This disease is chronic (chronic) and can not be cured. Although there is no cure, various treatments can reduce symptoms and improve the quality of life of people with the disease.
Additional explanation
The symptoms

Local scleroderma is the mildest scleroderma. This condition is often found in children, but can occur at any age. This type of scleroderma only affects the skin and causes hardening of one or more parts of the skin.

Symptoms of local scleroderma depend on the type:

• Morfea

Parts of the skin that become hard and tense are usually oval in shape
At first, this part appears as red or purple spots, then turns white in the middle.
Can occur in all parts of the body
Usually itchy
The part that hardens is shiny and has no hair
This condition can improve after several years and treatment may not be needed

• Morphea is generalized

Cutaneous patchs are similar to morphea, but in larger and more abundant amounts are found in the body
Changes in appearance (aesthetics) due to various skin colors caused by skin spots
Joint movement is limited due to thickening of the skin in the joint area.

• linearly

The skin becomes hard and tense in the form of lines on the arms, legs or face
Sometimes it can affect the underlying bones and muscles
This condition can improve after a few years, but can permanently inhibit growth. For example abbreviated members.

• In a sword stroke

A form of linear scleroderma that looks like a sword incision and appears on the face or scalp
Can cause atrophy (tissue loss) on the face, including the tongue and mouth
In rare cases, this condition is associated with abnormal facial bone growth, which results in facial deformity.

In systemic scleroderma, internal organs are also affected. This type is commonly found in women and in people aged 30 to 50 years. This type of scleroderma rarely occurs in children.

Symptoms that appear depend on the type of systemic scleroderma:

• located
• Milder symptoms
• Appears slow
• Affects facial skin, hands and feet
• Usually begins with Raynaud's disease (circulatory / circulatory disorders that cause vaginal discharge and cold fingers and hands)
• It can slowly damage the lungs, intestines or esophagus
• Trouble swallowing
• Esophageal reflux

• blend in
• Appears quickly
• Thickening of the skin on the abdomen, thighs, upper arms, hands and feet (can affect the whole body)
• Weight loss, fatigue, joint pain, and stiffness
• Can affect internal organs such as the heart, lungs, kidneys, and digestive system
• In cases of exposure to internal organs, it can be followed by shortness of breath, hypertension

The cause
The cause of scleroderma is still unknown. Scleroderma is thought to be caused by the activity of the immune system that is not controlled (the body's defenses), so that the production of collagen becomes excessive and uncontrolled. Collagen is a protein that forms connective tissue in the body, including the skin. Production and accumulation of excess collagen in connective tissue cells causes tissue fibrosis (formation of scar tissue and hardening). Hyperactivity of the immune system is thought to be related to certain genes.
diagnosis
To make a diagnosis of scleroderma, the doctor will ask for a family history of the disease, observe changes in skin thickness and perform several tests, such as:

• Take a small sample of the skin for a biopsy to determine whether there are abnormalities or not.
• Check respiratory function (to check lung function)
• X-rays to see bone disorders
• Lung scan
• Cardiac echocardiography

treatment

Scleroderma cannot be cured, but some drugs can help with the symptoms. The goals of Scleroderma treatment are to treat symptoms, prevent worsening of the disease, detect and treat complications, and maintain the function of parts of the body affected by scleroderma. Some people with mild scleroderma don't need treatment at all and some people may stop treatment when scleroderma is no longer active.

Current treatments are:

• Medication to improve blood circulation
• Medications to reduce the activity of the immune system and slow down the worsening of the disease
• Medication to prevent infection
• Steroids to treat joint and muscle disorders
• Moisturizer on the affected part to reduce itching
• Other medicines to treat other symptoms (pain, heartburn, reflux and high blood pressure)

Physiotherapy or occupational therapy can also help:

• Reduce pain
• Increase strength and mobility
• Maintain independence in daily activities

Surgery for scleroderma complications can also be used as a last resort.

Some operating options are:

• amputation

In cases of finger tissue death due to severe Raynaud's disease, an amputation must be performed.

• Lung transplantation

People with high blood pressure in the pulmonary veins may need a lung transplant.

Some other steps can help overcome the symptoms of scleroderma:

• Stay active

Exercise keeps the body supple, increases circulation, and reduces stiffness.

• Do not smoke

The nicotine in cigarettes causes narrowing or increased constriction of blood vessels, worsening Raynaud's disease. Narrowing of the arteries can also be permanent due to smoking.

• Overcome heartburn

To overcome these symptoms, avoid foods that cause bloating and avoid eating late at night. Lifting your head with a pillow during sleep can prevent reflux during sleep.

Protect the body from cold
Use warm gloves to protect your hands from cold exposure, especially when you carry items in the fridge.

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