WHAT IS SCLERODERMA

Scleroderma is a chronic connective tissue disease generally classified as an autoimmune rheumatic diseases.

The root of the word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. While the symptoms of scleroderma vary greatly for each person, hardening of the skin is one of the most visible manifestations of the disease. Scleroderma is not contagious, infectious, cancerous or malignant. The effects of scleroderma can range from very mild to life threatening. Scleroderma is a relatively rare disease—it’s estimated that approximately 300,000 Americans suffer from some form. Women are overwhelmingly more likely to suffer from this connective tissue disease. Currently, there is no cure but symptoms can be managed with treatment. 

Learn more at the Mayo Clinic

Types of Scleroderma

Localized Scleroderma

There are two kinds of localized scleroderma:

  • Morphea: This involves hard, oval-shaped patches on the skin. They start out red or purple and then turn whitish in the center. Sometimes, but not often, this type can affect blood vessels or internal organs. This is called generalized morphea.
  • Linear: This kind causes lines or streaks of thickened skin to form on the arms, legs, or face.

Systemic Scleroderma

There are two kinds of systemic scleroderma:

  • Limited Scleroderma: Typically mild, Limited Scleroderma only affects a small area of the body—usually just the fingers and face. It is more common among Caucasians than other populations. While every person with scleroderma is different and has a different pattern of symptoms, the CREST syndrome is a common set of symptoms.
    • C - Calcinosis, or calcium deposits under the skin and in tissues
    • R - Raynaud’s phenomenon
    • E - Esophageal Dysmotility, causing heartburn
    • S - Sclerodactyly, or thick skin on the fingers.
    • T - Telangiectasias, which are enlarged blood vessels (appear as red spots)
  • Diffuse SclerodermaOften more severe, Diffuse Scleroderma affects more areas of the skin. While symptoms vary, the most commonly affected areas include the skin of the arms, legs, and trunk. As a result, the tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes also an inflammation of the joints, tendons and muscles that accompanies the skin thickening. In severe cases, the tight skin on the face can reduce the size of a person’s mouth. The skin can lose or gain pigment; making areas of light or dark skin.  Some people lose hair on the limbs, sweat less, and develop dry skin because of skin damage.

Learn more at Johns Hopkins Scleroderma Center.

SCLERODERMA SYMPTOMS

Affected Areas

Some of the most common areas of the body affected by Scleroderma are:

  • Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs.
  • Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud's disease, this condition also occurs in people who don't have scleroderma.
  • Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients.
  • Heart, lungs or kidneys. Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-

Complications

Scleroderma symptoms range from mild to severe and may lead to some of the following medical complications:

  • Fingertips. The variety of Raynaud's disease that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). In some cases, gangrene and amputation may follow.
  • Lungs. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
  • Kidneys. When scleroderma affects your kidneys, you can develop elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
  • Heart. Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis). Scleroderma can also raise the pressure on the right side of your heart and cause it to wear out.
  • Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of dental decay increases even more.
  • Digestive system. Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus — as well as bouts of constipation alternating with episodes of diarrhea.

Learn more at the Mayo Clinic

A view of hands affected by Scleroderma .

DIAGNOSIS

Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose.

After a thorough physical exam, your doctor may suggest blood tests to check for elevated blood levels of certain antibodies produced by the immune system. He or she may remove a small tissue sample (biopsy) of your affected skin so that it can be examined in the laboratory for abnormalities.

Your doctor may also suggest breathing tests (pulmonary function tests), a CT scan of your lungs and an echocardiogram of your heart.

Learn more at the Mayo Clinic

TREATMENT & MEDICATIONS

Because everyone reacts to Scleroderma differently, identifying your disease subtype, stage, and involved organs is very important in determining the best course of action for treatment. Typically medications recommended for Scleroderma treatment focus on the four main features of the disease: inflammation, autoimmunity, vascular disease, and tissue fibrosis. While no two cases are identical, below lists some common treatment options:

  • Anti-Inflammatory Medications: there are two major types of inflammation that are related to the disease process. The first is a more conventional type that can cause arthritis (inflammation in the joints), myositis (inflammation in the muscles), or serositis [inflammation in the lining of the heart (pericarditis) or lining of the lung (pleuritis)]. This type of inflammation responds to traditional antiinflammatory drugs: NSAIDs (e.g. ibuprofen) or corticosteroids (e.g. prednisone). The other type of inflammation relates to the skin and other tissue injury caused by the scleroderma process. This phase of the disease does not appear to respond to NSAIDs or corticosteroids, although the exact role of corticosteroids is not fully studied. There are risks associated with the use of these agents, including gastrointestinal disease, fluid retention, and renal toxicity. 
  • Immunosuppressive Therapy: one of the most popular approach to controlling the inflammatory phase of scleroderma. This treatment focuses on the associated tissue damage and fibrosis caused by Scleroderma. There are several drugs that are being used, but only a few well designed studies have been performed. These immunosuppressing drugs include methotrexate, cyclosporine, antithymocyte globulin, mycophenolate mofetil and cyclophosphamide. A major area of current research is the use of aggressive immunosuppressive therapy either with very-high-dose cyclophosphamide or with autologous bone marrow transplantation.
  • Vascular Disease Drug Therapy: the vascular affects of scleroderma are widespread and affect medium and small arteries. The main result of this vascular affect is Raynaud's Disease, and there is evidence that repeated episodes of ischemia (low-oxygen state) occur in other tissues. Low blood flow into the skin and tissues is thought not only to damage tissue by the lack of nutrition and oxygen but to activate fibroblasts and promote tissue fibrosis. Therefore, treatment of the vascular disease is now considered crucial to controlling the disease as a whole as well as preventing specific organ damage. There are three major features of the vascular disease that potentially need treatment: vasospasm (spasm of blood vessels), a proliferative vasculopathy (thickening of blood vessels), and thrombosis (blood clots) or structural occlusion of the vessel lumen (blockage of blood vessels). Vasospasm is best treated with vasodilator therapy (drugs that open blood vessels). The most effective and popular vasodilator therapy continues to be the calcium channel blockers (e.g., nifedipine).
  • Anti-Fibrotic Agents: several drugs are used that have in vitro (in the tissue culture) ability to reduce collagen production or to destabilize tissue collagen. The older medications in this category include colchicine, para-aminobenzoic acid (PABA), dimethyl sulfoxide, and D-penicillamine. The search for new drugs that alter the fibrotic reaction is probably one of the most active areas of scleroderma research. Strategies include directly suppressing the fibroblast and its ability to make collagen, inhibiting the cytokines that activate the fibroblast, and the use of agents that might break down collagen faster and promote tissue remodeling.

Learn more at Johns Hopkins Scleroderma Center

SCLERODERMA SUPPORT

The following is placeholder text known as “lorem ipsum,” which is scrambled Latin used by designers to mimic real copy. Aliquam bibendum, turpis eu mattis iaculis, ex lorem mollis sem, ut sollicitudin risus orci quis tellus. In sit amet felis malesuada, feugiat purus eget, varius mi. Sed a ligula quis sapien lacinia egestas. In sit amet felis malesuada, feugiat purus eget, varius mi.

Scleroderma Foundation

The Scleroderma Foundation is the national organization for people with scleroderma and their families and friends. It was formed January 1, 1998, by a merger between the West Coast-based United Scleroderma Foundation and the East Coast-based Scleroderma Federation. The national office is headquartered in Danvers, Mass.