What Is Scleroderma?
Scleroderma, often called CREST syndrome, is a chronic and rare autoimmune condition characterized by replacing normal tissue with dense, thick, and fibrous tissue. In unaffected individuals, the immune system helps defend the body against bacteria, disease, and infection. In those with scleroderma, the immune system triggers other cells to produce an overabundance of collagen, a protein naturally found in the skin and body. This extra collagen is then deposited into the skin and the organs, resulting in the hardening and thickening of the tissue.
This process is similar to that of the scarring process on the skin. While scleroderma skin symptoms are most commonly reported, this can also affect many other body areas, such as the gastrointestinal tracts, lungs, kidneys, blood vessels, heart, muscles, and joints. In its most severe forms, scleroderma can be life-threatening. There are two primary types of scleroderma: localized and systemic. Systemic scleroderma can be divided into two main types: diffuse and limited.
Localized scleroderma is the more common form of the disease. It usually affects only a person's skin, typically appearing as waxy patches or streaks on the skin. This less severe form of scleroderma can dissipate or stop progressing without treatment, though it often requires treatment. Morphea scleroderma, a form of localized scleroderma, causes patches of the skin to thicken into firm, oval-shaped areas. These patches can have a yellow and waxy appearance, with the surrounding skin presenting with a red or bruise-like edge. Another form of localized scleroderma, linear scleroderma, causes lines of thickened, different-colored skin to run down the arm, leg, or forehead.
Systemic scleroderma, also known as systemic sclerosis, can develop quickly or gradually. As with localized scleroderma, systemic scleroderma can cause issues with internal organs. Many individuals with this form of the disease experience fatigue. Limited cutaneous scleroderma occurs gradually and usually affects the fingers, hands, face, lower arms, and lower legs.
It can also create issues with blood vessels and the esophagus. The limited form can affect the internal organs, but it is generally milder than diffuse scleroderma. Diffuse cutaneous scleroderma affects many parts of the body. Not only can it affect the skin, but it can also affect many different internal organs, potentially affecting digestive and respiratory functions as well as causing kidney failure. Systemic scleroderma can sometimes be serious and life-threatening.
Patients with limited scleroderma do not experience kidney issues. Generally, the thickening of the skin is limited to the fingers, hands, forearms, feet, and legs. Limited scleroderma is also called CREST syndrome, where each letter represents a characteristic disease factor.
- Calcinosis (abnormal calcium deposits in the skin)
- Raynaud's phenomenon (decreased blood flow to fingers)
- Esophageal dysmotility (difficulty swallowing)
- Sclerodactyly (skin tightening on the fingers)
- Telangiectasias (red spots on the skin)
According to the National Scleroderma Foundation, scleroderma is considered an autoimmune disorder that affects more than 50 million people in the United States. Scleroderma is typically more common in women than in men, usually occurring between the ages of 35 and 55, though a pediatric form can also occur. In addition to thickening the skin, the following scleroderma symptoms may occur in those with this disorder.
- Swelling on the feet and hands
- Red spots on the skin
- Excessive calcium deposits in the skin
- Joint contractures or rigidity
- Tight, mask-like facial skin
- Ulcerations on the toes and fingertips
- Stiffness and pain in the joints
- Persistent cough
- Shortness of breath
- Acid reflux
- Trouble with swallowing
- Digestive problems
- Gastrointestinal issues
- Weight loss
- Hair loss
The exact cause of scleroderma is unknown. In some cases, scleroderma can affect multiple people within one family. However, most cases do not show any family history of the disease. Scleroderma is not contagious, meaning you cannot catch it from anyone who has it, nor can you transmit it to another person. Research suspect that several factors may contribute to the disease, including genetic makeup, certain environmental factors, changes within the immune system, and hormones. For more information, please contact Advanced Dermatology and Skin Cancer Center.
How Is Scleroderma Diagnosed?
Achieving a scleroderma diagnosis is not always an easy process. Since scleroderma can affect the skin and other body parts, such as the joints, it can be initially mistaken for lupus or rheumatoid arthritis. After discussing your family medical history, your physician will perform a thorough physical examination.
Your dermatologist will look for any of the symptoms mentioned above, particularly the hardening of the skin around the fingers and toes or skin discoloration. If scleroderma is suspected, tests will be ordered to confirm the diagnosis and determine the disease's severity. These tests may include blood tests, pulmonary function tests, electrocardiograms, gastrointestinal tests, kidney function tests, and other diagnostic tests.
How Is Scleroderma Treated?
There is currently no cure for scleroderma, but scleroderma treatment is available and directed at helping patients control and manage their symptoms. Because this disease can produce various symptoms, a combination of approaches is often required to treat and manage the disease effectively. Available treatments may include skin treatments, digestive remedies, and treatment of lung disease (for patients with scleroderma and rapidly worsening pulmonary fibrosis). With regard to skin treatments for localized scleroderma, topical medications often prove beneficial. Special moisturizers are used to help the skin retain moisture as well as to treat hardened, scaly skin. To improve blood flow so sores can heal, nitrates may be prescribed. Nitrates relax the smooth muscles, which cause the arteries to widen or dilate. Smooth muscles generally form the support blood cells and several internal organs. Your specific scleroderma treatment plan will be determined by your dermatologist based on factors unique to your health and skin requirements.
Management Of Scleroderma
In addition to taking all prescribed recommendations correctly and as directed by your physician, there are several ways individuals diagnosed with scleroderma can better manage the disease. These include exercising regularly, protecting the joints and skin, modifying the diet, and effectively managing stress.
Exercising regularly will help improve your overall physical health and well-being, but it can also help you keep your joints flexible and improve your circulation. We recommend consulting your physician regarding the exercises appropriate for your needs.
Protecting your joints is a critical part of managing your scleroderma. When you experience joint pain, avoid lifting heavy objects or performing actions that can place strain on them and risk additional joint injury. Consult your doctor or physical therapist about protecting your joints.
Taking proper care of your skin and certain precautions can be beneficial in taking care of the dry, thickened skin patches resulting from localized scleroderma. There are many ways to accomplish this. You should dress appropriately during colder months. Keep your body and face warm and protected from the cold weather with winter-appropriate clothing, accessories, and footwear. This will help keep your circulation flowing, and your blood vessels open. You should wear multiple thin layers, as this will keep you warmer than a single, thick layer. Wear loose-fitting shoes to keep adequate blood supply to your feet and legs. Help your skin retain moisture by installing a humidifier in your living space. Last, use skin care products as recommended and as directed by your dermatologist.
In addition to consuming healthy foods that provide your body’s required amounts of vitamins and nutrients, you should eat foods that do not aggravate your scleroderma, or any resulting symptoms from the disease. You should avoid foods that cause heartburn, drink water to soften food further, eat a high-fiber diet, and eat smaller meals more regularly throughout the day.
The effects of stress can play a role in reducing your blood flow and it can also affect many other aspects of your life, including your emotional wellbeing and your health. As such, it is important to learn how to effectively manage or reduce stress. Potential solutions can include getting proper rest and sleep, avoiding stressful situations (when possible), eating a healthy diet, controlling anxieties and fears with healthy methods, and exercising regularly.