Scleroderma is an autoimmune disease that attacks connective tissue, causing it to thicken and harden. This disease can attack the skin, blood vessels, or organs, such as the lungs, kidneys, and heart.
Scleroderma is characterized by skin that is thick, hard, white, and slippery like wax. Scleroderma usually appears on the skin of the hands, feet or face. If not treated immediately, this disease can inhibit the activities of the sufferer and cause disturbances in the internal organs.
Causes of Scleroderma
Scleroderma occurs when the immune system, which is supposed to protect the body from infection, actually attacks the connective tissue. This condition triggers cells in the connective tissue to produce excess collagen . Collagen itself is a type of protein that forms connective tissue.
When collagen production increases dramatically, collagen will accumulate in the skin and organs. As a result, there is thickening and hardening of the skin and affected organs.
Although the exact cause of this condition is not known, there are a number of factors that are thought to increase a person's risk of developing scleroderma, namely:
- Female gender
- Age 35–55 years
- Having a family with scleroderma or an autoimmune disease
- Suffering from another autoimmune disease, such as lupus , rheumatoid arthritis , or Sjögren's syndrome
- Using chemotherapy drugs, such as bleomycin
- Continuous exposure to hazardous chemicals
Scleroderma Symptoms
Scleroderma can affect any part of the body. If scleroderma occurs only in certain areas of the skin, the condition is called localized scleroderma . Meanwhile, scleroderma that attacks the internal organs is called systemic sclerosis .
Localized scleroderma and systemic sclerosis can cause different complaints and symptoms. Here is the explanation:
Localised scleroderma
Localized scleroderma is the mildest type of scleroderma. This condition only occurs in the skin and is more common in children. Localized scleroderma is characterized by the appearance of one or more patches of thickened and hardened skin.
There are two types of localized scleroderma spots , namely morphoea and linear. Morphoea spots have the following characteristics:
- Oval-shaped
- About 2–20 cm in size
- Reddish or purplish in color, then turn whiter
- The surface of the spots is hairless and shiny like wax
- Feels itchy
- Can appear on any part of the body
- Spotting may improve in a few years without treatment
Meanwhile, linear spots have distinctive characteristics, such as:
- Shaped like a long line
- Appears on the skin of the face, head, legs or arms
- Gets better after a few years, although in some cases it may cause permanent shortening of a limb, such as an arm
In addition, the crusting of the linear patches can affect the underlying layers, such as the muscles or bones.
Systemic sclerosis
Systemic sclerosis is a type of scleroderma that doesn't only affect the skin, but also affects internal organs, such as the heart, lungs, kidneys, and digestive tract. This condition, which is more often experienced by women aged 30–50 years, 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 , namely pale fingertips or toes due to impaired blood flow, usually due to exposure to cold temperatures
- Calcinosis or accumulation of calcium in the body, one of the symptoms of which is the appearance of hard lumps under the skin ( calcinosis cutis )
- Telengiectasis, namely small blood vessels that grow and appear on the surface of the skin, and sometimes look like red spots
- Sclerodactyly , namely the skin on the fingers that is thin and looks tight making it difficult to move
- Esophageal dysmotility , namely movement disorders of the esophagus causing difficulty swallowing ( dysphagia )
The above symptoms may develop slowly and get worse over time. Limited scleroderma is considered milder than diffuse scleroderma. In diffuse scleroderma , complaints usually appear in the form of:
- Accumulation of collagen and hardening of connective tissue that occurs in organs, such as lungs, kidneys, heart, and digestive tract
- Hardening and skin changes all over the body
- Pain to stiffness in joints or muscles
- Weight loss
- Fatigue
- Hard to breathe
- Dry eyes or dry mouth
The symptoms of diffuse scleroderma may come on suddenly and get worse quickly in the first few years. Nonetheless, symptoms can be controlled and cured if treated appropriately.
When to see a doctor
Check with your doctor if you experience 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 . Meanwhile, if you have been diagnosed with scleroderma, undergo treatment given by a doctor regularly so that your condition is always monitored.
Diagnosis Skleroderma
To diagnose scleroderma, the doctor will ask about the symptoms experienced by the patient, as well as the medical history of the patient and his family. After that, the doctor will carry out a physical examination to identify any hardening or thickening of the skin.
Next, the doctor will carry out investigations to confirm the diagnosis and assess the severity of the scleroderma. The examination includes:
- Blood tests , to assess kidney function and measure levels of certain antibodies which are generally elevated when experiencing an autoimmune disease
- Biopsy by examining samples from the skin, to determine whether there is abnormal tissue
- Electrocardiography (EKG), to check the electrical activity of the heart which is generally disturbed if scleroderma has caused scarring in the heart
- Echocardiography (heart ultrasound), to get a picture of the condition of the heart and assess whether there are complications from scleroderma
- Scanning with a CT scan , to see the condition of the lungs or other internal organs
- Pulmonary function tests , to determine the performance of the lungs
- Endoscopy , to see the condition of the digestive tract, including the esophagus
Scleroderma Treatment
There is no medication that can cure scleroderma. Treatment is only limited to helping relieve symptoms, inhibiting the development of the disease, and preventing complications. Treatment methods that can be given are as follows:
Drugs
Several types of drugs that will be given to people with scleroderma are:
- Non-steroidal anti-inflammatory drugs (NSAIDs), to relieve pain and inflammation
- Corticosteroids in cream or tablet form, to help reduce inflammation in the joints, skin, and slow skin changes
- Immunosuppressants , to suppress the work of the immune system
- Vasodilators , to dilate blood vessels and improve blood flow to certain parts of the body, such as the fingers, lungs, and kidneys
- Proton pump inhibitors or antacids, to treat digestive disorders
- Antibiotics , to prevent and treat bacterial infections
Therapy
Physiotherapy or occupational therapy aims 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 a skin rash that doesn't go away.
Operation
In cases of severe scleroderma and causing complications, surgery can be performed. One of them is an amputation in a patient with Raynaud's syndrome who has gangrene in his finger.
Other types of surgery that can be done are surgical removal of hardened lumps under the skin and lung transplantation if scleroderma causes lung damage.
Apart from undergoing the treatment above, there are several efforts that can be made to help the recovery process. These efforts include:
- Exercise regularly
- Do not lift heavy objects
- Apply skin moisturizer
- Avoid foods that can increase stomach acid
- Eat healthy, nutritionally complete and balanced food
- Adequate sleep and rest
Scleroderma Complications
Untreated scleroderma can cause serious complications, such as:
- Tissue death in a finger or toe that is at risk of amputation
- Pulmonary hypertension and pulmonary fibrosis
- Kidney failure
- Hypertension
- Pericarditis , heart rhythm disturbances ( arrhythmias ), or heart failure
- Erectile dysfunction in men and vaginal dryness in women
- Gastrointestinal disorders, such as difficulty swallowing, constipation, or diarrhea
- Decreased saliva production so that teeth break down quickly
- Joint stiffness, thus interfering with movement
In pregnant women, scleroderma has a risk of causing miscarriage or premature birth .
Scleroderma Prevention
Scleroderma is difficult to prevent. 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-ups regularly, especially if you are frequently exposed to chemicals
In addition, if you have been diagnosed with scleroderma, follow the recommendations and treatment given by the doctor, adopt a healthy lifestyle, and identify and avoid things that can trigger complaints.