Scleroderma: 7 Warning Signs & Powerful Treatment Options

Scleroderma is an autoimmune disease that causes inflammation and fibrosis (thickening) in the skin and other areas of the body.

When an immune response tricks tissues into thinking they are injured, it causes inflammation, and the body makes too much collagen, leading to scleroderma. Too much collagen in your skin and other tissues causes areas of tight, hard skin. Scleroderma may involve many systems in your body.

There are two major types of scleroderma:

Localized scleroderma only affects the skin and the structures directly under the skin.
Systemic scleroderma, also called systemic sclerosis, affects many systems in the body. This is the more serious type of scleroderma and can damage your blood vessels and internal organs, such as the heart, lungs, and kidneys. This subset is also divided into two additional categories called “limited” and “diffuse,” which represent how much skin involvement there is in the body.

There is no cure for scleroderma. The goal of treatment is to relieve symptoms and stop the progression of the disease. Early diagnosis and ongoing monitoring are important.

The cause of scleroderma is unknown. However, researchers think that the immune system overreacts and causes inflammation and injury to the cells that line blood vessels. This triggers connective tissue cells, especially a cell type called fibroblasts, to make too much collagen and other proteins. The fibroblasts live longer than normal, causing a buildup of collagen in the skin and other organs, leading to some of the signs and symptoms of scleroderma. There can also be injuries to blood vessels.

Anyone can get scleroderma; however, some groups have a higher risk of developing the disease. The following factors may affect your risk.
Sex. Scleroderma is more common in women than in men.
Age. The disease usually appears between the ages of 30 and 50 and is more common in adults than in children.
Race. Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely. For example:
The disease is more common in African Americans than European Americans.
African Americans with scleroderma develop the disease earlier when compared with other groups.
African Americans are more likely to have more skin involvement and lung disease when compared with other groups.

Localized scleroderma occurs more commonly in children but can also appear in adults. It generally appears in one or both of these patterns:
Morphea, or patches of scleroderma that may be half an inch or larger in diameter.
Linear scleroderma is characterized by the thickening of the skin in a line. This usually extends down an arm or leg, but sometimes runs down the forehead and face.
Systemic scleroderma, sometimes called systemic sclerosis, affects your skin, tissues, blood vessels, and major organs. Doctors usually divide systemic scleroderma into two types based on the degree of skin involvement:
Limited cutaneous scleroderma, which comes on gradually and affects the skin on your fingers, hands, face, lower arms, and legs below the knees.
Diffuse cutaneous scleroderma, which comes on more rapidly and starts as being limited to the fingers and toes, but then extends beyond the elbows and knees to the upper arms, trunk, or thighs. This type usually has more internal organ damage.

The symptoms of scleroderma vary from person to person, depending on the type of scleroderma you have.

Localized scleroderma typically causes patches of thick, hard skin in one of two patterns.

Morphea causes patches of skin to thicken into firm, oval-shaped areas. These areas may have a yellow, waxy appearance surrounded by a reddish or bruise-like edge. The patches may stay in one area or spread to other areas of skin. The disease usually becomes inactive over time, but you may still have darkened patches of skin. Some people also develop fatigue (feeling tired).
Linear scleroderma causes lines of thickened or differently colored skin to run down your arm, leg, and, rarely, on the forehead.
Systemic scleroderma, also known as systemic sclerosis, may come on quickly or gradually and may also cause problems with your internal organs in addition to the skin. Many people with this type of scleroderma have fatigue.
Limited cutaneous scleroderma comes on gradually and usually affects the skin on your fingers, hands, face, lower arms, and legs below the knees. It often causes problems with your blood vessels and esophagus. The limited form has less frequent major internal organ involvement, such as kidney disease or progressive lung disease, but it is generally milder than in the diffuse form.
Diffuse cutaneous scleroderma comes on suddenly, usually with skin thickening on your fingers or toes. The skin thickening then spreads to the rest of your body above the elbows and/or knees. This type can damage your internal organs, such as:
- - Anywhere along your digestive system.
  - Your lungs.
  - Your kidneys.
  - Your heart.

Researchers do not know the exact cause of scleroderma, but they suspect that several factors may contribute to the disease:

Genetic makeup. Genes can increase the chance for certain people to develop scleroderma and play a role in determining the type of scleroderma they have. You cannot inherit the disease, and it is not passed from parent to child like some genetic diseases. However, first-degree relatives of people with scleroderma are at a higher risk of developing scleroderma than the general population.
Environment. Researchers suspect that exposure to some environmental factors, such as some chemicals, may trigger scleroderma.
Immune system changes. Abnormal immune or inflammatory activity in your body triggers cell changes that cause the production of too much collagen. In some cases, an immune reaction to developing cancer cells may trigger scleroderma.
Hormones. Women develop most types of scleroderma more often than men. Researchers suspect that hormonal or immunological differences between women and men might play a part in the disease.

It can be difficult for doctors to diagnose scleroderma because the symptoms vary from person to person and are similar to other diseases. There is no single test to diagnose the disease; instead, doctors use a combination of the following to help diagnose scleroderma. Your doctor may:

Ask about your medical history.
Ask about your current and past symptoms.
Perform a physical exam.

Your doctor may recommend additional testing, such as:

Ordering laboratory tests to check for certain antibodies that mistakenly target and react to your own tissues. Some of the antibodies may be common in people with scleroderma. However, antibodies may develop due to other factors, so a blood test alone does not diagnose scleroderma.
Performing a skin biopsy.

To look for problems with internal organs, such as the heart, lungs, or kidneys, your doctor may order additional testing. Early diagnosis of organ involvement helps doctors treat and manage the disease. Testing may include:

Computerized tomography (CT) uses a scanner to take images of the lungs and other organs.
An echocardiogram, which uses sound waves to create moving pictures of your heart.
Pulmonary function tests measure the function of the lungs.

Most people will see a rheumatologist for scleroderma treatment. A rheumatologist is a doctor who specializes in rheumatic diseases such as arthritis and other inflammatory or autoimmune disorders. Dermatologists, who specialize in conditions of the skin, hair, and nails, may also play an important role in treating the disease, particularly for people with localized scleroderma.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors providing your care. These health care providers may include:

Cardiologists, who specialize in treating diseases of the heart and blood vessels.
Dental providers can treat complications from the thickening of tissues of the mouth and face.
Gastroenterologists, who treat digestive problems.
Mental health professionals provide counseling and treat mental health disorders such as depression and anxiety.
Nephrologists, who treat kidney disease.
Occupational therapists teach how to safely perform activities of daily living.
Orthopaedists treat and perform surgery for bone and joint diseases or injuries.
Primary care providers, including physicians, nurse practitioners, and physician assistants.
Physical therapists teach ways to build muscle strength.
Pulmonologists treat lung disease and problems.
Speech-language pathologists specialize in the treatment of speech, communication, and swallowing disorders.

Depending on the type of scleroderma you have and your symptoms, living with the disease may be hard. To help, try to take an active part in treating your scleroderma. The following tips and suggestions may help.

Keep warm. Your body regulates its temperature through the skin. So, dress in layers, wear gloves and socks, and avoid cold rooms and weather when possible.
Try to avoid cold or wet environments that may trigger Raynaud’s phenomenon symptoms.
If you smoke, quit. Nicotine and smoking cause blood vessels to contract, which can make some symptoms worse and cause lung problems.
Apply sunscreen before you go outdoors to protect against further damage from the sun’s rays.
Use moisturizers on your skin to help lessen stiffness.
Use humidifiers to moisten the air in your home in colder winter climates. Clean humidifiers often to stop bacteria from growing in the water.
Avoid hot baths and showers, as hot water dries the skin.
Avoid harsh soaps, household cleaners, and caustic chemicals. Wear rubber gloves if you use such products.
Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.
Visit the dentist regularly for check-ups.
Reach out to online and community support groups.
Keep the lines of communication open. Talk to your family and friends to help them understand the disease.
Talk to a mental health professional for help with coping with a chronic illness.

Some types of scleroderma can affect parts of the digestive system. Doctors may prescribe heartburn, constipation, and motility medications to help manage these symptoms. Here are some tips to help if you have digestive symptoms:

Eat small, frequent meals.
After meals, stay upright for 3 hours. Try to avoid reclining or slouching after eating.
Eat moist, soft foods, and chew them well. If you have difficulty swallowing or if your body doesn’t absorb nutrients properly, your doctor may prescribe a special diet.
Drink less alcohol and caffeine.
Stay hydrated.
When it is time to sleep, raise the head of your bed with blocks or use a wedge pillow. Using several pillows is not as helpful as raising the head of the bed by using blocks or special wedges.

Your treatment depends on the type of scleroderma you have, your symptoms, and which tissues and organs are affected. Treatment can help control the symptoms and limit damage.

Your doctor may recommend medications, including:

Anti-inflammatory medications to manage pain and reduce swelling.
Topical creams to treat skin changes, including tightness and itching.
Immunosuppressants, which may suppress the overactive immune system, can help control some aspects of the disease. Your doctor may prescribe oral, IV, or injectable immunosuppressants.
Vasodilators help blood vessels dilate (widen), which may treat Raynaud’s phenomenon and some lung issues.

In addition, your doctor may prescribe medications that are typically approved to treat other rheumatic diseases that have similar symptoms to scleroderma.

Many people benefit from physical or occupational therapy to:

Relieve pain.
Improve muscle strength and mobility, including muscles in your arms, legs, and jaw.
Teach you techniques to help with activities of daily living. For example, if hand pain and stiffness make it hard to brush your teeth, a therapist can recommend toothbrushes and devices to make flossing easier.

Regular dental care is important because scleroderma can make your mouth dry and damage connective tissues in your mouth, speeding up tooth decay and causing your teeth to become loose. Tightening facial skin can also make your mouth opening smaller and narrower, which makes it harder to care for your teeth. Here are some ways to avoid tooth and gum problems:

Brush and floss your teeth regularly.
Have regular dental checkups. Contact your dentist immediately if you experience mouth sores, mouth pain, or loose teeth.
Talk to your dentist and doctor about the best methods for you to use to keep your mouth moist.
Use special mouthwashes or toothpastes for dry mouth. You can also talk to your doctor about medications that treat dry mouth.

Lung Damage

Almost all people with systemic scleroderma have some loss of lung function. Some people develop severe lung disease, which comes in two forms:

Pulmonary fibrosis is a hardening or scarring of lung tissue because of excess collagen.
Pulmonary hypertension is high blood pressure in the arteries that carry blood from the heart to the lungs.

Treatment differs for these two conditions:

Pulmonary fibrosis may be treated with medications that suppress the immune system or medications that can help counter fibrosis.
Pulmonary hypertension may be treated with medications that dilate the blood vessels.

To help minimize lung complications, work closely with your doctor.

Watch for signs of lung disease, including fatigue, shortness of breath, dry cough, or difficulty breathing, and swollen feet. Report these symptoms to your doctor.
Follow up regularly with your doctor for evaluation of your lung function. This may include standard lung function tests, which measure your lung volumes to monitor the course of lung fibrosis. Checking for pulmonary hypertension early helps doctors manage and treat the condition, even before you may notice symptoms.
Get regular flu and pneumonia vaccines as recommended by your doctor, especially if you are taking immunosuppressant medications or have lung disease.

Heart Problems

Some people may develop complications that cause heart problems, including:

Cardiomyopathy, scarring, and weakening of the heart.
Myocarditis is an inflammation of the heart muscle.
Arrhythmia, abnormal heartbeat.

Treatments for heart complications can range from medications to surgery and vary depending on the nature of the condition.

Kidney Problems

Renal crisis is uncommon but can be serious for people with systemic scleroderma. Renal crisis happens when blood pressure levels rise suddenly to dangerous levels, which can quickly lead to kidney failure. Side effects of certain medications, such as corticosteroids, can also trigger renal crisis. It is important that you and your doctor work together to monitor your blood pressure, including:

Check your blood pressure regularly, and let your doctor know if you have any new or different symptoms, such as a headache or shortness of breath. If your blood pressure is higher than usual, call your doctor right away.
If you have kidney problems, remember to take your medications as prescribed. In the past several decades, medications known as ACE (angiotensin-converting enzyme) inhibitors have made scleroderma-related kidney failure a less threatening problem than it used to be.

Because scleroderma can affect many different organs and organ systems, you may have several different doctors providing your care. These health care providers may include:

Cardiologists, who specialize in treating diseases of the heart and blood vessels.
Dental providers can treat complications from the thickening of tissues of the mouth and face.
Gastroenterologists, who treat digestive problems.
Mental health professionals provide counseling and treat mental health disorders such as depression and anxiety.
Nephrologists, who treat kidney disease.
Occupational therapists teach how to safely perform activities of daily living.
Orthopaedists treat and perform surgery for bone and joint diseases or injuries.
Primary care providers, including physicians, nurse practitioners, and physician assistants.
Physical therapists teach ways to build muscle strength.
Pulmonologists treat lung disease and problems.
Speech-language pathologists specialize in the treatment of speech, communication, and swallowing disorders.

Keep warm. Your body regulates its temperature through the skin. So, dress in layers, wear gloves and socks, and avoid cold rooms and weather when possible.
Try to avoid cold or wet environments that may trigger Raynaud’s phenomenon symptoms.
If you smoke, quit. Nicotine and smoking cause blood vessels to contract, which can make some symptoms worse and cause lung problems.
Apply sunscreen before you go outdoors to protect against further damage from the sun’s rays.
Use moisturizers on your skin to help lessen stiffness.
Use humidifiers to moisten the air in your home in colder winter climates. Clean humidifiers often to stop bacteria from growing in the water.
Avoid hot baths and showers, as hot water dries the skin.
Avoid harsh soaps, household cleaners, and caustic chemicals. Wear rubber gloves if you use such products.
Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.
Visit the dentist regularly for check-ups.
Reach out to online and community support groups.
Keep the lines of communication open. Talk to your family and friends to help them understand the disease.
Talk to a mental health professional for help with coping with a chronic illness.

Eat small, frequent meals.
After meals, stay upright for 3 hours. Try to avoid reclining or slouching after eating.
Eat moist, soft foods, and chew them well. If you have difficulty swallowing or if your body doesn’t absorb nutrients properly, your doctor may prescribe a special diet.
Drink less alcohol and caffeine.
Stay hydrated.
When it is time to sleep, raise the head of your bed with blocks or use a wedge pillow. Using several pillows is not as helpful as raising the head of the bed by using blocks or special wedges.

Source:
National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Scleroderma.
https://www.niams.nih.gov/health-topics/scleroderma

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