Scleroderma, also known as systemic sclerosis, is a chronic, although rare, autoimmune disease in which normal tissue is replaced with dense, thick fibrous tissue characterized by hardening and tightening of the skin.
Normally, the immune system helps defend the body against disease and infection. In patients with scleroderma, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in the skin and organs, which causes hardening and thickening It may also cause problems in the blood vessels, internal organs and digestive tract.
Scleroderma is a group of rare diseases that more often affects women. It commonly occurs between the ages of 30 and 50.
The symptoms can affect many parts of your body. They include –
• Hardened or thickened skin that looks shiny and smooth. It’s most common on your hands and face.
• Raynaud’s phenomenon
• Ulcers or sores on your fingertips
• Small red spots on your face and chest
• Firm, oval-shaped patches on your skin
• Trouble swallowing
• Painful or swollen joints
• Muscle weakness
• Dry eyes or mouth (Sjogren’s syndrome)
• Swelling, mostly of your hands and fingers (edema)
• Shortness of breath
• Belly cramps and bloating
• Weight loss with no clear cause
There are two kinds of scleroderma –
▪︎ Localized scleroderma mainly affects your skin. It happens in one of two forms.
• Morphea. This involves hard, oval-shaped patches on your skin. They start out red or purple and then turn whitish in the center. Sometimes, this type can affect blood vessels or internal organs. This is called generalized morphea.
• Linear. This kind causes lines or streaks of thickened skin on your arms, legs, or face.
▪︎ Systemic scleroderma, also called generalized scleroderma, can involve many body parts or systems. There are two types:
• Limited scleroderma. This comes on slowly and affects the skin of your face, hands, and feet. It can also damage your lungs, intestines, or esophagus, the tube that carries food from your mouth to your stomach. It’s sometimes called CREST syndrome, after its five common signs:
• Calcinosis. This is when calcium salts form nodules under your skin or in your organs.
• Raynaud’s phenomenon. This is a lack of blood flow to parts of your body such as your fingers, toes, or nose, usually because of cold. Your skin might turn red, white, or blue.
• Esophageal dysfunction. This is when your esophagus doesn’t work the way it should.
• Sclerodactyly. This is a thickening of the skin. It usually causes problems with moving your fingers and toes.
• Telangiectasia. This is when small blood vessels grow near the surface of your skin.
▪︎ Diffuse scleroderma. This comes on quickly. Skin on the middle part of your body, thighs, upper arms, hands, and feet can become thick. This form also affects internal organs like your heart, lungs, kidneys, and gastrointestinal tract.
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body’s connective tissues, including your skin.
Doctors don’t know exactly what causes this process to begin, but the body’s immune system appears to play a role.
Risk Factors –
Anyone can get scleroderma, but it does occur much more often in women than in men. Several combined factors appear to influence the risk of developing scleroderma –
People who have certain gene variations appear to be more likely to develop scleroderma. This may explain why a small number of scleroderma cases appear to run in families and why some types of scleroderma are more common for certain ethnic groups.
• Environmental triggers.
Research suggests that, in some people, scleroderma symptoms may be triggered by exposure to certain viruses, medications or drugs. Repeated exposure, such as at work, to certain harmful substances or chemicals also may increase the risk of scleroderma. An environmental trigger is not identified for most people.
• Immune system problems.
Scleroderma is believed to be an autoimmune disease. This means that it occurs in part because the body’s immune system begins to attack the connective tissues. People who have scleroderma may also have symptoms of another autoimmune disease — such as rheumatoid arthritis, lupus or Sjogren’s syndrome.
Your doctor will do a physical exam and ask about your health history. They might order tests including –
• Imaging tests such as X-rays and CT scans
• Blood tests
• Gastrointestinal tests
• Lung function tests
• Heart tests such as EKGs and echocardiograms
Treatment can’t cure the condition, but it can help reduce symptoms and slow disease progression. Treatment is typically based on a person’s symptoms and the need to prevent complications.
Treatment for generalized symptoms may involve:
• immunosuppressants, such as methotrexate or Cytoxan
• nonsteroidal anti-inflammatory drugs (NSAIDs)
Depending on your symptoms, treatment can also include:
• blood pressure medication
• medication to aid breathing
• physical or occupational therapy
• regular dental care to prevent damage to your mouth and teeth
Ayurvedic Perspective –
In Ayurveda, Scleroderma is termed as Uttana Vatarakta. It afflicts the superficial tissues which comprise skin & muscles. It appears to be Kushta (skin disease) with skin lesions and muscle pain. It is caused due to aggravation of Vata dosha due to intake of improper foodstuffs or long-distance rides on animals. Further, the Rakta gets vitiated due to intake of lavana, amla, katu, kshara, etc., and blocks the passage of Vayu. The Rakta dhatu vitiated by Vayu burns the whole blood in the body, which then gravitates towards the foot, thus leading to Uttana Vatarakta.
Ayurvedic treatment of Scleroderma involves treatments that involve the elimination of the root cause of the condition. This involves therapies of Lepa, Abhyanga, Parisheka, Avagahana, and Upanaha. The treatment also involves Shodhana therapy & Shamana therapy.
Ayurvedic Medication –
Along with these external therapies, internal medication in the form of a combination of herbs is also given. This includes Vasadi Kwatha, Navakarshika Kwatha, Guduchi Kwatha, Kasmaryadi Kwatha, Patoladi Kwatha, Kokilakshyadi Kwatha, Laghu Manjishtadi Kwatha, etc. This line of treatment helps in the reversal of the Scleroderma condition in a holistic way.
In addition to taking prescribed medications correctly and regularly, there are many steps a person with scleroderma can take to better manage the disease. These include –
Regular exercise will not only help improve your overall physical and spiritual well-being, but it will also help keep your joints flexible and improve circulation. Consult your doctor or physical therapist for appropriate exercises.
▪︎ Joint protection
When your joints hurt, avoid lifting heavy objects or performing chores that may place a strain on them, thus risking further injury. A physical therapist can help you learn new ways to perform daily activities without placing undue strain on your joints.
▪︎ Skin protection
Taking proper precautions and care of your skin can be beneficial not only for symptoms of Raynaud’s phenomenon, but also in taking care of the dry, thick patches of skin that result from localized scleroderma. There are many ways to accomplish this, including:
• During the colder months, be sure to dress appropriately. Keeping your body warm and protected from the cold weather with boots, a hat, gloves and a scarf will help keep the blood vessels in your extremities open and your circulation flowing.
• Wear multiple thin layers. These will keep you warmer than wearing one thick layer.
• Wear loose-fitting boots or shoes to keep the blood supply moving to your feet.
• Put a humidifier in your house to help keep the air moist.
• Use soaps and creams that are designed especially for dry skin.
Aside from eating healthy foods to get the proper amounts of vitamins and nutrients, it is important to eat foods that do not aggravate existing stomach problems. Ways to do this include –
• Avoiding foods that cause heartburn.
• Drinking water or another liquid to soften food further.
• Eating high fiber foods to cut down on constipation.
• Eating more, smaller meals as opposed to three large meals. This enables the body to digest the food more easily. If you eat a large meal, wait at least four hours before lying down.
• Raising the head of your bed about six inches by placing blocks or bricks underneath it. This will prevent stomach acid from entering the esophagus while you are sleeping.
▪︎ Dental care
For those patients with scleroderma who also have Sjögren’s syndrome, proper dental care is essential. Sjögren’s syndrome increases the risk of developing cavities and tooth decay.
▪︎ Stress management
Because the effects of stress can play a part in reducing your blood flow, as well as affect many other aspects of your emotions and health, it is important to learn to manage or reduce stress by having proper sleep, healthy diet, practicing meditation etc.
Although no cure has been found for scleroderma, the disease is very often slowly progressive and manageable, and people who have it may lead healthy and productive lives. Like many other conditions, education about scleroderma and local support groups can be the greatest tools for managing the disease and reducing the risk of further complications.
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