A rare autoimmune disease characterized by tightening and hardening of the skin and underlying connective tissues supporting the body is termed as scleroderma. It can be observed in anyone irrespective of sex, age and geographical area. However, women and those in the 30-50 age groups are at increased risk for the disease. Exact cause of the disease is unknown but the body immune system is responsible for all scleroderma symptoms. Increased exposure to silica dust, paint thinners, and use of certain chemotherapy drugs increases the risk for scleroderma.
Types of scleroderma
There are two main categories of scleroderma localized and systemic scleroderma.
- Localized scleroderma affecting only the skin is of two types Morphea and Linear scleroderma. Morphea is characterized by presence of thick patches of skin with purple border and white in the middle. These patches may develop at different regions of the body. Thickened patches observed in Morphea disappear after three to five years. Linear scleroderma develops only on one side of the body and is more common in children. It is characterized by presence of bands of hardened skin on arms, legs and forehead.
- Systemic scleroderma affects not only the skin even the underlying organs and blood vessels are involved. It is of two types, limited scleroderma and diffused scleroderma. Limited scleroderma starts gradually affecting the skin of feet, hands and face. It may also result in damage to intestines, lungs and esophagus. Diffuse scleroderma develops rapidly and causes thickening of skin of trunk, hands, feet and upper arm. It may also affect internal organs such as kidneys, lungs and heart.
Symptoms of scleroderma
Distinct scleroderma symptoms are observed depending on the organ system being involved. Symptoms also vary among different patients. Commonly observed scleroderma symptoms
- Raynaud’s phenomenon or white fingers disease: Blood vessels of the extremities get constricted resulting in pain, numbness and even change in color of toes and fingers. Patients with this phenomenon respond severely to low temperatures and are also easily prone to emotional distress.
- Gastroesophageal reflux disease: Acid reflux from the stomach causes damage to the esophagus at the point where it attaches to the stomach. Poor movement of food materials through the intestines also results in poor absorption of nutrients.
- Changes in skin: Skin becomes tight in the regions around the fingers, hand and face. As a result the patient feels hard to move the affected area. Skin also looks shiny. Increased production of collagen stimulated by the immune system is responsible for thickening of the skin.
- Difficulty to swallow: Poor functioning of the muscles of the esophagus often results in swallowing difficulties and reflux of stomach acids in to esophagus resulting heartburn and scarring of the esophageal tissue.
- Small white colored bumps under the skin: Small bumps are calcium deposits. They generally in elbows, fingers and in knees. These deposits are tender and are at high risk of getting infected.
- Digestive problems: Poor or reduced muscle activity of the intestines causes digestive problems such as heartburn, diarrhea, delayed flow of foods in the intestine and trouble swallowing.
- Discoloration of the skin is observed due to poor blood circulation.
- Tiny red spots develop on chest, palms, lips, tongue and face due to dilated tiny blood vessels. These spots are often painless.
- Increased blood pressure: Thickening of blood vessels elevates blood pressure in pulmonary and systemic arteries.
- Shortness of breath due to lung or heart damage or due to increased pressure in the arteries of lungs.
- Ulcers on finger tips.
- Painful and swollen joints.
- Tingling or numbness of the skin. Skin may also become puffy.
- Reduced ability of skin to stretch.
- Fingers may get curled.
- Loss of normal functionality of the hand due to tightening of the muscles.
- Weight loss.
- • Edema.
Limited scleroderma symptoms
Five symptoms are specifically observed in limited scleroderma or the CREST syndrome.
- Calcinosis: deposition of calcium in skin.
- Raynauds phenomenon.
- Esophageal dysfunction due to scarring of the tissue of the esophagus.
- Sclerodactyl characterized by tightening of the skin of toes and fingers.
- Telangiectasias characterized by red spots on face, lips and tongue.
As feared by many scleroderma symptoms are not contagious. However, they hurt your self esteem and interfere in accomplishing routine tasks. One should seek medical advice if signs such as development of ulcers on finger tips, thickening of skin, increased sensitivity to cold, red spots on face and lips and swelling of extremities are observed to prevent complications.
Treatment of scleroderma
There is no permanent cure for scleroderma. However, scleroderma symptoms can be alleviated to reduce the risk of complications by using appropriate treatment strategy.
Before starting the treatment process presence of scleroderma should be confirmed as scleroderma symptoms are similar to those commonly observed in other similar diseases. Diseases such as atherosclerosis, polycythemia and other neurologic disorders also result in symptoms similar to Raynaud’s phenomenon. Nail fold capillary test is often used as a best indicator for scleroderma. Skin under the finger nails is observed for the presence of small blood vessels. Disappearance of small blood vessels in feet and hands is a primary indication for scleroderma. It is further confirmed by physical examination, X-ray, blood tests and skin biopsy. Organs such as esophagus, lungs and heart are also evaluated.
Scleroderma symptoms are relieved by using a combination of therapies. Treatment plan for scleroderma aims at relieving the pain and improving the blood circulation to the organs.
- Non steroidal anti-inflammatory drugs such as ibuprofen are used to relieve pain and inflammation.
- Corticosteroid medications or moisturizers are applied topically to treat tightened skin condition. Colchicine is helpful in reducing inflammation of the skin.
- Immune suppressants such as methotrexate and cyclophosphamide are used to relieve joint and muscle problems. These drugs reduce impact of overreacting immune system on internal organs also.
- Drugs such as vasodilators are used to improve blood circulation to extremities i.e. hands, feet, fingers. Topical nitroglycerin works best when applied on the sides of affected fingers or toes.
- Medications such as angiotensin converting enzyme inhibitors are used to regulate blood pressure and heartburn. They also help to alleviate kidney and lung problems. Drugs such as omeprazole, esmeprazole also help to reduce esophagus irritation and heartburn. Avoiding smoking and caffeine containing products also helps to alleviate heartburn. Avoiding smoking also saves you from developing lung cancer.
- Regular practice of physical workouts and practicing measures to cope up with stress improves overall health.
- Special diet, drugs and antibiotics may be used to relieve digestive disorders such as constipation and diarrhea caused by bacteria.
- Physiotherapy is used to reduce stiffness of joints and tissues. By reducing pain and improving strength, physiotherapy aids patient in performing routine activities and remain independent.
- Use of gloves and socks helps to remain warm and reduce some of the scleroderma symptoms.
- Orthopaedic surgery is used to correct worse joints and skin deformities. Cosmetic surgeries such as use of ultraviolet light and laser surgery are used to get rid of skin lesions or to camouflage them.
- Anti-rheumatic drugs such as hydroxychloroquinone help to slow down the progress of disease.
- Acupuncture: This treatment is suggested to alleviate blood circulation problems, heal the ulcers in finger tips and even reduce development of fibrous tissue.
- Organ transplantations are performed if possible to replace badly damaged organs. For example, lung transplantation is performed in patients with severe pulmonary hypertension. Fingers are amputated if gangrene develops due to finger ulcers.
People with limited scleroderma can continue to lead quality life without problems by using medications, taking well balanced diet with low fat, taking plenty of fluids and by avoiding caffeine, tobacco and alcohol. To prevent permanent damage of vital organs such as heart it is essential to keep pressure in arteries under control by using timely medications and regular checkups. However, people with diffuse scleroderma find it hard managing the disease and survive for about 10 to 15 years after the onset of the disease.
Scleroderma : Prevention and precautions
It is not possible to prevent scleroderma. However, those suffering with scleroderma symptoms can reduce the risk of infections such as pneumonia and other bacterial infections by taking appropriate vaccinations. It is very important to take the annual flu vaccine and the pneumococcal vaccine.