What is Scleroderma?
Systemic sclerosis, also referred to as scleroderma, is a group of diseases that cause the skin to tighten and harden. Additionally, it might affect the digestive system, internal organs, and blood vessels.
Only the extent of skin involvement can classify scleroderma as "limited" or "diffuse." Any additional vascular or organ problems might be present in any form. Skin alone is the sole organ affected by localised scleroderma, sometimes called morphea.
Scleroderma has no known cure, although therapies can reduce symptoms, halt progression, and enhance quality of life.
Types of Scleroderma
There are two kinds of scleroderma:
- Localized scleroderma
- Systemic scleroderma
Symptoms of Scleroderma
Depending on which regions of the body are affected, scleroderma manifests itself differently in each individual.
Skin-related symptoms and signs
A hardness and tightness of the skin is a common symptom of scleroderma.
Typically, the face, fingers, hands, and feet are the first bodily parts to be impacted. Forearms, upper arms, the chest, belly, lower legs, and thighs can all experience skin thickening in certain persons. Itching and swelling are examples of early signs. Because of the tightness, affected skin may turn lighter or darker in colour and appear glossy. Other symptoms are:
- Swelling of the hands and feet
- Red spots on the skin
- Excessive calcium deposition in the skin
- Joint rigidity
- Tight, mask-like facial skin
- Ulcerations on the fingertips and toes
- Pain and stiffness in the joints
- Persistent cough
- Shortness of breath
- Heartburn(acid reflux)
- Difficulty swallowing
- Digestive and gastrointestinal problems
- Constipation
- Weight loss
- Fatigue
- Hair loss
When to see a doctor?
Not getting treatment for Scleroderma will decrease the capacity to breathe and its progression leads to lung tissue scarring. Additionally, the arteries leading to your lungs may experience elevated blood pressure. An abrupt rise in blood pressure and quick kidney failure are symptoms of a dangerous kidney condition known as scleroderma renal crisis. Though, there is no specific warning signal for Sclerodema, any changes in the skin accompanied with other symptoms should not be ignored. Meeting a doctor will help in correlation of varying symptoms, go with suggestive tests and conclude any medical condition.
Collagen is overproduced and builds up in body tissues, causing scleroderma. The connective tissues in your body, including your skin are made of a fibrous kind of protein called collagen. This collagen production starts increasing.
Although doctors are unsure of the precise trigger for this process, the immune system of the body seems to be involved. Scleroderma most likely results from a mix of immune system issues, genetics, and environmental stressors.
Risk Factors of Scleroderma
Scleroderma can affect everyone, but women are far more likely to develop it than men are. Scleroderma risk appears to be influenced by a number of interrelated variables, including:
- Genetics :
Scleroderma appears to be more common in people who have specific gene variants. This may help to explain why some cases of scleroderma seem to run in families and why some ethnic groups are more likely to develop specific kinds of the disease.
- Changes in the environment :
According to research, exposure to specific viruses, medicines may cause scleroderma symptoms in some individuals. Scleroderma risk may also be increased by repeated exposure to some dangerous agents or chemicals, such as those present at work. For the majority of persons, there is no known environmental trigger.
- Immune system problems :
It is thought that scleroderma is an autoimmune condition. It follows that it happens in part as a result of the body's immune system attacking the connective tissues. Scleroderma patients may also exhibit signs of other autoimmune diseases, such as lupus, rheumatoid arthritis, or Sjogren's syndrome.
Diagnosis of Scleroderma
Scleroderma can be challenging to diagnose since it affects many different body parts and can manifest in so many different ways.
Your doctor may recommend blood testing to check for high levels of specific antibodies produced by the immune system following a complete physical examination.
To assist identify whether your digestive system, heart, lungs, or kidneys are impacted, your doctor may also recommend additional blood testing, imaging, or organ-function tests.
Scleroderma Treatment
The excessive collagen production which is a defining feature of scleroderma cannot be reversed or treated. However, a number of treatments can aid with symptom control and avert consequences.
Medications
The selection of treatment will vary depending on the symptoms of scleroderma because it can affect many different body parts.
- Dilate blood vessels :
It can be treated with blood vessel-dilating, blood pressure medicines.
- Immune system suppression :
Some scleroderma symptoms, such as skin thickening or worsening lung damage, may be slowed down by immune-suppressing medications, such as those prescribed after organ transplants.
- Decreasing digestion symptoms :
Heartburn can be relieved by taking pills to lower stomach acid. Bloating, diarrhoea, and constipation may be lessened by antibiotics and drugs that facilitate the movement of food through the intestines.
- Stop infections :
Regular immunizations against the flu and pneumonia can aid in protecting lungs that have already suffered damage from scleroderma.
- Reduce pain :
Your doctor may recommend prescription painkillers if over-the-counter medicines are ineffective.
Therapies
You can preserve your independence with daily duties while increasing your strength and mobility with the aid of physical or occupational therapy. Hand treatment might help avoid contractures of the hands.
Surgical methods
For those whose severe symptoms have not improved with more conventional therapy, stem cell transplants may be a possibility. Organ transplants may be considered if the lungs or kidneys have suffered significant damage.
Lifestyle changes and selfcare
Following lifestyle changes will help one to manage this disease and decrease its severity:
- Keep moving :
Exercise keeps your body flexible and helps to reduce stiffness and enhances circulation. Exercises that increase range of motion can keep your skin and joints flexible. This is crucial at all times, but it's crucial in the beginning stages of the condition.
- Keep your skin safe :
Apply lotion and sunscreen frequently to care for dry or tight skin. Avoid taking hot baths or showers using harsh soaps, or using household chemicals on your skin because these things might irritate and dry out your skin even more.
- Avoid smoking :
Raynaud's illness is exacerbated by nicotine because it causes blood vessels to constrict. Additionally, smoking can lead to persistent blood vessel narrowing and worsening of lung conditions. Ask your doctor for advice if you're having trouble quitting smoking.
- Prevent heartburn :
Foods that cause heartburn or gas should be avoided. Avoid eating after midnight as well. To prevent stomach acid from backing up into your oesophagus while you sleep, raise the head of your bed. Symptom relief may be provided by antacids.
- Don't expose yourself to the cold :
When your hands are exposed to the cold, such as when you reach into a freezer, put on a warm pair of gloves for protection. In order to lessen the symptoms of Raynaud's disease, it's crucial to maintain a warm core body temperature. Wear layers of warm clothing, protect your face and head, and wear warm footwear when you're outside in the cold.
Do's and Don’ts
This condition requires proper treatment and a set of do’s and don’ts to be followed to manage it and its related symptoms and infections. During the treatment and even after the treatment, one needs to follow these:
| Do’s |
Don’ts |
| Take enough sleep for at least 7 to 9 hours. |
Forget to clean affected skin. |
| Eat healthy food and avoid junks. |
Forget to take medications. |
| Regular exercises or yoga advised by the therapist. |
Forget to cover your skin while going out. |
| Protect your skin and take good care of it. |
Forget to go for routine checkups. |
| Take precautions to avoid any kind of infection. |
Do smoking. |
| Keep the thickened skin or sores warm. |
Go for cosmetic procedures that tighten your skin or boost your skin's collagen levels. |
Follow the above tips and immediately inform the doctor if there are new pain or symptoms.
Care at Medicover Hospitals
At Medicover Hospitals, we have the most trusted team of doctors and medical experts who are experienced in providing excellent healthcare services to the patients with compassionate care. Our diagnostic department is equipped with modern technology and equipment to conduct the tests required for the diagnosis of Scleroderma, based on which a dedicated treatment plan is designed. We have an excellent team of dermatologists and rheumatologists who diagnose and treat this condition with utmost precision that brings successful treatment outcomes.
Citations
Scleroderm 10.1056/NEJMra0806188
The Heart in Scleroderma
Treatment of Scleroderma
