Scleroderma is an autoimmune disease that attacks the connective tissue, causing it to thicken and harden. This condition can occur in the skin, blood vessels, and organs, such as the lungs, kidneys, and heart.
Scleroderma can be characterized by the appearance of thick, hard, white skin, and looks slippery like wax. This condition can appear on the hands, feet, or face. Scleroderma that attacks the skin in addition to disturbing appearance can also interfere with movement.
Causes of Scleroderma
Scleroderma occurs when the immune system, which is supposed to protect the body from injury and infection, attacks the connective tissue. This condition triggers the cells in the connective tissue to produce collagen (a type of protein that forms connective tissue) in excessive amounts.
When collagen production increases dramatically, collagen will accumulate in the skin and organs. As a result will appear hardening and thickening of the skin and organs.
Although the exact cause of this condition is not known, there are a number of factors that are thought to make a person more susceptible to developing scleroderma, namely:
- Female gender
- 35–55 years old
- Have a family suffering from scleroderma or autoimmune disease
- Have another autoimmune disease, such as lupus, rheumatoid arthritis, or Sjogren's syndrome
- Using chemotherapy drugs, such as bleomycin
- Continuous exposure to hazardous chemicals, such as silica dust
Symptoms of Scleroderma
Scleroderma can affect any part of the body. If scleroderma only occurs in certain areas of the skin, the condition is called localized scleroderma, while scleroderma that attacks deeper organs is called systemic sclerosis.
These two conditions can cause different complaints and symptoms. The following is an explanation of the two conditions:
Localized scleroderma
Localized scleroderma This is the mildest type of scleroderma. This condition only occurs on the skin and is more common in children. This type of scleroderma is characterized by the appearance of one or more patches on the skin that are thickened and hardened.
There are two types of hard spots in this condition, namely: morphoea and linear. The morphoea spots have the following characteristics:
- Oval shaped
- Spots vary in size from 2–20 cm
- Initially red or purple, then the spots turn whiter
- The surface is not covered with hair and shiny like wax
- Usually itchy
- It can occur in any part of the body
- Spots can improve in a few years without treatment
Linear spotting similar to morphoea, but are distinguished by the following points:
- Shaped like a long line
- Commonly occurs on the skin of the face, head, legs, or arms
- Hardening of the skin can affect the underlying layers, such as muscles or bones
- Can improve after a few years, although in some cases it can cause permanent shortening of limbs, such as the arms
Systemic sclerosis
Systemic sclerosis is a type of scleroderma that not only occurs in the skin, but also affects some internal organs, such as the heart, lungs, kidneys, and digestive tract. This condition is more often experienced by women aged 30-50 years and is divided into two types, namely: limited scleroderma and diffuse scleroderma.
Limited scleroderma causes hardening of the connective tissue in the skin of the face, hands, and feet, as well as in the blood vessels and parts of the digestive system. This condition is characterized by:
- Raynaud's phenomenon or syndrome, which is the blanching of the tips of the fingers or toes due to lack of blood flow, usually due to exposure to cold temperatures
- Calcinosis, which is a buildup of calcium in the body, one of the symptoms is the appearance of hard lumps under the skin (calcinosis cutis)
- Telengiectasis, which is small blood vessels that grow and appear on the surface of the skin (sometimes they look like red patches)
- Sclerodactyly, namely the skin on the fingers that thins and looks tight so it is difficult to move
- Esophageal dysmotility, which is a movement disorder in the esophagus, so that it can be difficult to swallow (dysphagia)
The above symptoms develop slowly and worsen over time. Limited scleroderma milder than diffuse scleroderma. On diffuse scleroderma, a complaint will appear in the form of:
- Collagen buildup and connective tissue hardening occur in organs, such as the lungs, kidneys, heart, and digestive tract
- Hardening and changes of skin all over the body
- Pain to stiffness in joints or muscles
- Weight loss
- Fatigue
- Hard to breathe
- Dry eyes or dry mouth
Symptoms diffuse scleroderma occurs suddenly and worsens rapidly in the first few years. However, with proper treatment, symptoms can be controlled and cured.
When to go to the doctor
Check with your doctor if you experience any of the symptoms mentioned above. Early examination is needed so that the condition can be treated immediately and the risk of complications can be prevented.
If you have risk factors, such as having a family with scleroderma or suffering from another autoimmune disease, have regular medical check-ups. It is necessary to monitor your health condition.
If you have been diagnosed with scleroderma, undergo treatment given by your doctor regularly, so that your condition can be monitored.
Scleroderma Diagnosis
To diagnose scleroderma, the doctor will ask the patient's symptoms, as well as the patient's and family's medical history. After that, the doctor will perform a physical examination to identify any hardening or thickening of the skin.
Next, the doctor will perform the following investigations to confirm the diagnosis and assess the severity of scleroderma:
- Blood tests, to measure levels of certain antibodies that are generally elevated during autoimmune disease and to assess kidney function
- Biopsy by taking samples from the skin, to determine the presence or absence of abnormal tissue
- Electrocardiography (ECG), to determine the electrical activity of the heart which is generally disturbed if scleroderma has caused scar tissue in the heart
- Echocardiography (ultrasound of the heart), to describe the condition of the heart and to assess the presence or absence of complications from scleroderma
- Scanning with a CT scan, to determine the condition of the lungs or other internal organs
- Lung function tests, to find out how well the lungs are working
- Endoscopy, to see the condition of the gastrointestinal tract, including the esophagus
Scleroderma Treatment
There is no cure for scleroderma. Treatment aims to help relieve symptoms, inhibit disease progression, and prevent complications. Treatment can be done in the following ways:
Drugs
Some types of drugs that will be given to patients with scleroderma are:
- Non-steroidal anti-inflammatory drugs (NSAIDs), to relieve pain and inflammation
- Corticosteroid drugs in the form of creams or tablets, to help reduce inflammation in the joints, skin, and slow changes in the skin
- Immunosuppressant drugs, to suppress the work of the immune system
- Vasodilator drugs, to dilate blood vessels and facilitate blood flow to certain body parts, such as the fingers, lungs, or kidneys
- Proton pump inhibitors or antacids, to treat indigestion
- Antibiotic drugs, to prevent and treat bacterial infections
Therapy
Physiotherapy or occupational therapy for scleroderma sufferers is done to treat pain, increase body strength and flexibility, and train patients to adapt to their limited range of motion.
Another therapy that can be given is light therapy such as laser therapy. These therapies aim to treat damage to the skin, such as hardened patches or skin rashes that don't go away.
Operation
In cases of severe and complicated scleroderma, surgery can be performed. One of them is finger cutting surgery in patients with Raynaud's phenomenon who have experienced gangrene on his finger.
Other types of surgery that can be done are surgical removal of hardened lumps under the skin, to lung transplants in people with severe lung damage.
Complications of Scleroderma
Some of the complications that can occur due to scleroderma are:
- Tissue death in fingers or toes at risk of amputation
- Pulmonary hypertension and pulmonary fibrosis
- Kidney failure
- Hypertension
- Pericarditis, arrhythmias (heart rhythm disturbances), or heart failure
- Erectile dysfunction in men and vaginal dryness in women
Scleroderma Prevention
There is no specific prevention for scleroderma. However, there are several things that can be done to reduce the risk of developing scleroderma, namely:
- Check with your doctor regularly if you have certain risk factors, such as suffering from an autoimmune disease or having a family with scleroderma
- Do medical check-up periodically when exposed to chemicals, such as silica dust
In addition, if you have been diagnosed with scleroderma, follow the advice and treatment given by the doctor, adopt a healthy lifestyle, and identify and avoid things that can trigger complaints.