Scleroderma is an autoimmune condition that involves hardening of the skin and connective tissues, leading to either temporary or permanent cell damage. The disease may lead to improper functioning of any affected limbs, as well as the heart, lungs, or kidneys. Direct causes of the overactive immune response remain unknown.
The word “scleroderma” comes from the Greek words sclero, or hard, and derma, or skin.
This chronic disease is not a malignant or contagious condition, and is not restricted to the skin’s surface as it can affect the tissues and blood vessels within major organs and joints.
There are varying degrees and types of scleroderma, which can lead to serious and life-threatening complications.
Scleroderma (Hardening of the Skin) Causes
Although a direct cause of scleroderma has not been found, researchers have discovered that both genetics and the environment may play a role, each in their own way.
As scleroderma stems from the build-up of collagen (specifically scar tissue following an injury), the immune system response may be the initiator.
Furthermore, one study of the group with the largest number of scleroderma cases, Choctaw Indians, indicated that a heredity factor is responsible. It also suggested that environmental factors may be involved.
This, and other studies, illustrate that environmental pollutants such as solvents, epoxy resins, or pesticides may contribute to the development of scleroderma.
What Are the Types of Scleroderma?
Scleroderma can be divided into two major groups, each with its own subgroup.
1. Localized Scleroderma
This is a mild form of the condition, with the skin becoming inflamed from the excess collagen. Localized scleroderma commonly affects the skin and the muscles. Two forms of localized scleroderma are separated by their physical characteristics.
This type appears near the waistline as patches of skin with a smooth but hard and waxy surface. These vary in size, location, color, and may come and go.
It is common among children and adults between 20 and 50 years of age.
This scleroderma may first appear as a band or straight line of thin, hard, and waxy skin. Affecting the surface and deep layers of the skin, it is usually seen on the forehead, leg, or arm in childhood and may cause stunted growth in a limb.
An easy way to recognize linear scleroderma is by the sword shape it forms, which gives it the familiar name of en coup de sabre.
2. Systemic Scleroderma
Known as systemic sclerosis, this form of scleroderma is found in various parts of the body’s connective tissue. This can include the blood vessels, joints, and muscles of the heart, lungs, kidneys, stomach, bowels, and esophagus, as well as the skin’s tissue.
Sclerosis refers to hardening and this change can affect the actions of the tissues. It can appear as either diffuse scleroderma or limited scleroderma. Both can lead to life-threatening conditions.
Diffuse scleroderma can happen suddenly in one or several locations of the body. The affected area may be inflamed and present an expanded, hard surface on the skin. This form of systemic scleroderma may affect the organs.
Limited scleroderma involves thicker skin patches that appear mainly on the face, hands, and fingers over time. While it is less serious because the organs are not often involved, the condition pulmonary hypertension can arise.
Limited may be referred to as CREST syndrome as it represents calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia.
The signs and symptoms of scleroderma vary in accordance to the location and severity of the tissue affected.
The affected patches of skin will appear tight with a hard, shiny, and tough surface. This can restrict the skin’s movement.
As a single incidence or grouping, the skin patches may be in a line or oval-shaped.
The fingers or toes may be painful and numb and possibly discolored. It may be a response to emotional stress or extremely cold temperatures.
3. Digestive System
Damage to the muscles within the gastrointestinal system may cause digestive issues such as acid reflux and the inability to properly absorb essential nutrients.
4. Heart, Lungs, Kidneys
Damaged, or scarred, tissues in major organs such as the heart, lungs, or kidneys may impair their functioning of these organs.
Scleroderma has symptoms and signs that may mimic other disorders and diseases. Proper diagnosis may require a series of lab tests, as well as a review of your medical history and physical examination.
An early sign of scleroderma may be Raynaud’s disease. It is distinguished by discoloration of the fingers or toes.
A blue, red, or white tint to the skin color after exposure to frigid temperatures is caused by a lack of blood flow. This disease leads to pain, numbness, and lesions and can be a separate skin issue or appear together with scleroderma.
Other factors your doctor will take note of include:
- Patches of tight and thickened skin, which may have a shiny surface or be swollen, particularly on the face; this change in skin tissue may affect the functioning of the affected area
- Spider veins, or telangiectasias that can often be seen on the skin’s surface; these lines, found commonly on the face, hands, and surrounding the nail beds, are blood vessels that have become enlarged
- Calcium salt deposits within the skin’s tissue
- Scleroderma affecting the tissues of the kidneys, which may cause high blood pressure
- Digestive issues including bloating, constipation, and swallowing difficulties
- Shortness of breath indicating tissue damage within the chest and lungs
- Joint pain as a result of damage to the connective tissue of the affected area
While there is no one blood test for scleroderma, examination of the blood may indicate any abnormalities such as antibody markers.
A sample of the affected skin tissue may be extracted for laboratory examination.
Pulmonary function tests to measure lung capacity and a computed tomography scan of the lungs may be necessary, and an echocardiogram will allow examination of heart function, blood flow, and any damage to the structures.
Doctors may perform a thermography test to measure blood flow within the blood vessels as well as the temperature of the tissue.
Soft tissue issues may be detected by use of an ultrasound scan or the use of a magnetic resonance imaging, or MRI, test.
Treatment of scleroderma focuses on the symptoms as there is no particular medication or therapy to stop or reverse the thickening of the skin tissue.
Mild cases may fade over several years while tissue damage within organs may progress over time.
Medications are used to:
- Dilate blood vessels to prevent complications of the kidneys, lungs, and to treat Raynaud’s disease
- Control the immune system to reduce symptoms
- Alleviate stomach acid to treat acid reflux
- Prevent infection of the lungs and the Raynaud’s disease complication of lesions
- Treat pain caused by inflammation
Therapy undertakes pain management and mobility issues caused by scleroderma. Occupational and physical therapy may help to improve strength while redirecting movement for daily activities.
Extreme cases of scleroderma may require surgery.
Lesions caused by Raynaud’s disease may lead to gangrene, which requires amputation of the affected digit to prevent spread of the infection.
A lung transplant may be necessary if the lungs show high blood pressure readings within the arteries, known as pulmonary hypertension.
Scleroderma treatment may include lifestyle changes such as regular physical activity to address joint pain and stiffening, improve circulation, and promote skin flexibility.
Symptoms of heartburn may be managed by eliminating gas-inducing foods from the diet, resting with the head and chest elevated, and avoiding overeating.
Smoking may also produce scleroderma symptoms as it promotes the constriction of blood vessels. Counseling to help patients stop smoking may be part of the treatment plan.
Covering the exposed skin from extremely cold temperatures may help prevent, or alleviate, scleroderma issues.
Who Is at Risk of Scleroderma?
Scleroderma is considered a rare disease with 300,000 patients within the United States. Of this number, women seem to be more susceptible to this chronic disease.
Genetics may be involved as it affects more African Americans and Native Americans than Caucasians. It is not considered to be a shared condition among family members, although a scleroderma patient often has family members with other autoimmune diseases.
Children are more apt to be diagnosed with localized scleroderma, while adults tend to have the systemic condition.
What Are the Complications of Scleroderma?
- Fingertips: Reduced blood flow to the fingertips may lead to permanent tissue damage. Lesions and ulcers may develop and be susceptible to gangrene infection, which can lead to amputation of the affected digit.
- Lungs: Scleroderma in the lungs may cause scarring of the lung tissue. This can impair functioning and lead to breathing difficulty. Pulmonary hypertension may also arise, which is high blood pressure within the arteries of the lungs.
- Kidneys: High blood pressure, an increase in protein, and scarring of the tissue in the kidneys may lead to kidney failure.
- Heart: Damage to the tissue of the heart may lead to an irregular heartbeat, congestive heart failure, and an increase in the pumping function of the right side, causing the heart to stop. Pericarditis may also be present. This sees the inflammation of the membranous sac.
- Teeth: Scleroderma reduces the production of saliva, leading to tooth decay. It also causes the skin to tighten, which can lead to narrowing of the facial surface, affecting the mouth. This may cause cleaning of the teeth to be difficult.
- Digestion: Tissue damage within the digestive system, including the esophagus, may lead to difficulty swallowing, promote acid reflux, and improper absorption. This may cause constipation, diarrhea, abdominal pains, and decrease appetite.
- Sexual Function: With connective tissues being affected by scleroderma, women may experience vaginal wall constriction and a reduction in natural sexual lubricant. Men may be affected with erectile dysfunction.
Scleroderma is characterized by patches of tightened and hardened skin tissue due to the immune system attacking healthy cells. This chronic disease may be difficult to diagnosis as it can affect the internal connective tissue of the organs and joints.
Depending on the type of scleroderma, symptoms can mimic other health conditions and diagnostic testing may be extensive.
The appearance of skin tightness may fade over time. Treatment of the symptoms may involve medications, physical therapy, and changes to lifestyle habits. Severe cases may require surgery.
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