Scleroderma is an uncommon skin condition that causes blood vessel and internal organ problems as well as the hardening and thickness of the skin. In the disorder known as scleroderma, the immune system attacks the connective tissue that lines blood vessels, internal organs, and other body cavities.
Scleroderma is a rare autoimmune connective tissue disease that causes the hardening and thickening of the skin. But the disorder isn’t confined to skin changes. It can affect your:
- Blood vessels
- Digestive system
Scleroderma is a condition in which the immune system attacks the connective tissue beneath the skin, as well as the tissue around internal organs and blood vessels. This results in scarring and tissue thickening in certain locations.
There are numerous forms of Scleroderma with varying degrees of severity. Some varieties are benign and may resolve on their own, while others can lead to serious and life-threatening complications.
Scleroderma has no treatment, yet most people who have it can live a full and active life. Scleroderma symptoms can usually be treated with a variety of therapies.
It has an annual incidence rate of four cases per million, a prevalence of 30 instances per million, and a female predominance of 4:1 in the United Kingdom. It can affect people of any age range, however, it is more common in people between 30 to 50.
Scleroderma, progressive systemic sclerosis, and CREST syndrome are all terms for systemic sclerosis. The acronym “CREST” stands for:
- Raynaud’s phenomenon
- Oesophageal dysmotility (oesophagal dysmotility – in the USA)
What are the types
What are the types of Scleroderma?
Scleroderma is divided into two types:
Affects only your skin is known as localised scleroderma. It manifests itself in one of two ways:
- Morphea. This is characterised by hard, oval-shaped spots on the skin. They begin crimson or purple and eventually fade to a pale colour in the middle. This type can sometimes harm blood vessels or internal organs. Generalized morphea is the term for this condition.
- Linear. This type causes thicker skin lines or streaks on your arms, legs, or face.
Scleroderma that affects multiple body parts or systems is known as systemic scleroderma or generalised scleroderma. There are two kinds of them:
This is a gradual onset condition that affects the skin of your face, hands, and feet. It can also harm your lungs, intestines, and oesophagus, which is the tube that connects your mouth to your stomach. It’s also known as CREST syndrome, after the five symptoms that it exhibits:
- Calcinosis. Calcium salts create nodules under your skin or in your organs when this happens.
- Raynaud’s syndrome. A condition in which the blood vessels in the hands and feet become cold A lack of blood flow to some portions of your body, such as your fingers, toes, or nose, is caused by a cold. It’s possible that your skin will turn red, white, or blue.
- Dysfunction of the oesophagus. This occurs when your oesophagus does not function properly.
- Sclerodactyly. This is a skin thickening condition. It frequently makes it difficult to move your fingers and toes.
- Telangiectasia. This occurs when little blood vessels form near the skin’s surface.
This happens quickly. Thick skin can develop on the centre of your body, thighs, upper arms, hands, and feet. Internal organs such as the heart, lungs, kidneys, and gastrointestinal tract are also affected by this type.
What are the symptoms
WHAT ARE THE SYMPTOMS OF SCLERODERMA?
In the early stages of the disease, Scleroderma may simply affect the skin. Around your lips, nose, fingers, and other bony places, you may notice your skin thickening and glossy spots appearing.
As the illness worsens, you may have restricted movement in the affected areas. Other signs and symptoms include:
- Hair thinning
- Calcium deposits, often known as white lumps under the skin, are a common occurrence.
- Under the skin’s surface, there are small, dilated blood vessels.
- Joint discomfort
- Breathing problems
- A cough that is dry
- Swallowing problems
- Oesophagal reflux disease
- Swelling in the abdomen after eating
The blood vessels in your fingers and toes may begin to spasm. When you’re chilly or under a lot of emotional stress, your extremities may turn white and blue. Raynaud’s phenomenon is the name for this condition.
What Causes Scleroderma?
Normally, the immune system of the body fights off any pathogens that infiltrate it. It reacts in this way to everything it doesn’t recognise in the body, then calms down after the infection is gone.
Scleroderma is hypothesised to develop when a portion of the immune system becomes overactive and out of control. This causes connective tissue cells to produce too much collagen, resulting in scarring and tissue thickening (fibrosis).
It’s unclear why this occurs. It’s considered that certain genes are involved, and having a close relative with the disease may raise your risk.
How is Scleroderma diagnosed?
During a physical examination, your doctor can detect Scleroderma-related skin abnormalities.
Scleroderma-related kidney abnormalities might lead to high blood pressure. Blood tests such as antibody testing, rheumatoid factor, and sedimentation rate may be ordered by your doctor.
Other diagnostic tests that may be used include:
- An X-ray of the chest
- A urinalysis is a test that looks at your urine to see if you
- A pulmonary CT scan (Scan of the lungs)
- Gastrointestinal tests
- Lung function tests
- Heart tests such as EKGs and echocardiograms
They may also take a small sample of skin (called a biopsy) to examine under a microscope by a specialist.
What are some Scleroderma
What are some Scleroderma risks factors?
The following are risk factors that can enhance your chances of having the disease:
- Being of Native American descent
- Being of African-Caribbean ancestry
- Being a woman
- Using certain chemotherapy drugs such as Bleomycin
- Being exposed to organic solvents and silica dust
Other than reducing risk factors that you can manage, there is no proven strategy to prevent Scleroderma.
What is the treatment
options for Scleroderma?
What is the treatment options for Scleroderma?
Although treatment won’t make Sleraderma go away, it will help to lessen the symptoms and stop the disease’s progression. Treatment is usually determined by a person’s symptoms as well as the need to avoid consequences.
Treatment options for nonspecific symptoms include:
- Immunosuppressive drugs like methotrexate and Cytoxan
- NSAIDs (nonsteroidal anti-inflammatory medicines) like ibuprofen or aspirin. They can help with swelling and pain.
- Steroids and other drugs can trigger your immune system to become sluggish. These can aid in the treatment of muscle, joint, and internal organ issues.
- Medications to boost blood flow to your fingers
- Medication to help open up the blood vessels in your lungs and to keep the tissue from scarring
- Medication for heartburn
- Medications to assist in moving food through your intestines
Treatment may involve the following, depending on your symptoms:
- Medicine for high blood pressure
- Breathing assistance medicine
- Physical therapy
- Light therapy, such as ultraviolet A1 phototherapy
- Nitroglycerin ointment to treat localized areas of tightening of the skin
- Increase the amount of fibre and water in your diet
- Occupational therapy
- Stress management
- If your organs are seriously damaged, you may need an organ transplant
To keep healthy with scleroderma, undertake lifestyle adjustments such as quitting smoking, staying physically active, and avoiding foods that cause heartburn.
Alternative and Complementary therapies used
when treating Scleroderma patients
Hyperbaric oxygen therapy
Hyperbaric oxygen therapy
What are the Potential
Complications of Scleroderma?
What are the Potential Complications of Scleroderma?
Complications that may occur as a result of treatment include:
- In your lungs, you have high blood pressure.
- In your lungs, there is scar tissue.
- The blood supply to your fingers and toes is cut off.
- Inflammation of the muscles
- Failure of the kidneys
Scleroderma treatments have vastly improved in the last 30 years. Despite the fact that there is no cure for Scleroderma, there are a variety of therapies available to assist you to manage your symptoms. If any of your symptoms are interfering with your regular life, consult your doctor. They can collaborate with you to make changes to your treatment plan.
You should also ask your doctor to assist you in finding local Scleroderma support groups. It can be easier to cope with a chronic ailment if you talk to other individuals who have had similar experiences.