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What You Need To Know About Scleroderma

Scleroderma is a range of disorders in which the skin and connective tissues tighten and harden. It is a long-term, progressive disease. This means it gradually gets worse.

Scleroderma is a rheumatic disease and a connective tissue disorder. It is also an autoimmune condition, in which the body's own immune system attacks the body's tissues.

This causes an overproduction of collagen, the protein that forms the basis of connective tissue. The result is a thickening, or fibrosis, and scarring of tissue.

Scleroderma is not contagious. It may run in families, but it often occurs in people without any family history of the disease. It ranges from very mild to potentially fatal.

It affects 75,000–100,000 people in the United States, mostly women aged 30–50 years. However, it is often more severe in men.

According to the American Academy of Dermatology Association, the Choctaw Native Americans in southeastern Oklahoma have the highest risk of developing scleroderma on the skin and in the internal organs.

In addition, in the U.S., 70% of people who get scleroderma are African American.

Additionally, some research suggests that African American people tend to develop scleroderma earlier and have more severe symptoms. In particular, they may have more symptoms relating to the skin or the lungs.

Scleroderma means "hard skin."

Early symptoms of scleroderma include changes in the fingers and hands, such as stiffness, tightness, and puffiness, due to sensitivity to cold or emotional stress.

There may be swelling in the hands and feet, especially in the morning.

Overall symptoms of scleroderma include:

calcium deposits in connective tissues

a narrowing of the blood vessels to the hands and feet, known as Raynaud's disease

problems of the esophagus, which links the throat and stomach

tight, thickened skin on the fingers

red spots on the face and hands

Scleroderma can also cause changes in skin pigmentation, including patches of skin that appear darker or lighter than a person's natural skin color.

Raynaud's disease can also cause the fingers or toes to become paler in the cold. However, this can be challenging to diagnose in people with darker skin as it may not always be easily recognizable.

However, symptoms will vary according to the type and how it affects the person, and whether it affects one part of the body or a whole body system.

There are two main types of scleroderma, including localized and systemic scleroderma.

Localized scleroderma mainly affects the skin, but it may have an impact on the muscles and bones.

Systemic scleroderma affects the whole body, including the blood and internal organs, and especially the kidneys, esophagus, heart, and lungs.

Localized scleroderma

Localized scleroderma is the mildest form. It does not affect the internal organs. There are two main types: morphea and linear scleroderma.

Morphea: Symptoms include oval-shaped patches of skin that are darker or lighter than a person's natural skin color and which may be itchy, hairless, and shiny. The shapes have a purple border and they are white in the middle.

Linear scleroderma: There may be bands or streaks of hardened skin on the limbs, and rarely the head and face. It can affect bones and muscles.

Systemic scleroderma

Systemic scleroderma affects the circulation of the blood and the internal organs.

There are two main types:

Limited cutaneous systemic sclerosis

This is the least severe type of systemic scleroderma. It affects the skin on the hands, feet, face, and lower arms and legs. There may be problems with the blood vessels, lungs, and digestive system.

Doctors sometimes call it CREST syndrome because the symptoms form the acronym, CREST:

C: calcinosis, or calcium deposits in tissues and under the skin

R: Raynaud's disease

E: esophageal problems, including GERD

S: sclerodactyly, or thick skin on the fingers

T: telangiectasias, or enlarged blood vessels, manifesting as red spots

The first sign is often Raynaud's disease, in which blood vessels narrow in the hands and feet, leading to circulatory problems in the extremities. People may experience numbness, pain, and color changes in response to stress or cold.

The skin on the hands, feet, and face may start to thicken.

The impact on the digestive system can lead to difficulty swallowing and gastroesophageal reflux disease (GERD), or acid reflux.

The intestinal muscles may be unable to move food through the intestines properly, and the body may not absorb nutrients.

Some of these symptoms can have other underlying causes, and not everyone who has Raynaud's disease or GERD will have scleroderma.

Diffuse systemic sclerosis

In diffuse systemic sclerosis, the skin thickening usually affects the area from the hands to above the wrists. It also affects the internal organs.

People with systemic types of scleroderma may experience weakness, fatigue, difficulty breathing and swallowing, and unintentional weight loss.

Doctors do not know what causes scleroderma, but they believe it to be an autoimmune condition that makes the body produce too much connective tissue. This leads to thickening, or fibrosis, and scarring of tissue.

Connective tissue forms the fibers that make up the framework that supports the body. They exist under the skin and around the internal organs and blood vessels, and they help support muscles and bones.

Genetic factors possibly play a role, and environmental factors too, but researchers have not confirmed this definitively.

People with scleroderma often come from families in which another autoimmune disease exists.

Scleroderma may be hard to diagnose because it develops gradually and appears in different forms, and because some of the symptoms, such as GERD, can occur in people without scleroderma.

A primary physician will carry out a physical examination and some tests and may refer a person to a rheumatologist, which is a specialist in joint and connective tissue diseases. A person may also need to see a dermatologist, which is a doctor specializing in skin diseases and conditions.

The following tests may be necessary:

looking at the skin under a dermatoscope to check for changes in the tiny blood vessels, or capillaries, around the fingernails

skin biopsy

blood tests to assess the levels of certain antibodies

The doctor will look for signs of skin thickening, GERD, shortness of breath, joint pain, and calcium deposits. They may also check for lung, heart, or gastrointestinal complications.

There is currently no cure for scleroderma, and no medication can stop the overproduction of collagen. However, doctors can treat organ system complications to minimize damage and maintain functionality.

Localized scleroderma may resolve on its own. Some medications can help control the symptoms and prevent complications.

The aim is to relieve the symptoms, prevent the condition from worsening, or at least slow it down, detect and treat complications as soon as possible, and minimize disabilities.

Treatment depends on how the disease affects the person:

Blood pressure medication may help dilate the blood vessels. This can reduce problems with the organs, such as the lungs and kidneys, and help to treat Raynaud's disease.

Immunosuppressants can calm or suppress the immune system.

Physical therapy may help manage pain, improve mobility, and improve strength. Aids, such as splints, can help with daily tasks.

Ultraviolet light therapy and laser surgery may help improve the condition and the appearance of the skin.

Calcium channel blockers and bisphosphonates treat calcinosis.

Hyaluronidase injections treat microstomia, which can happen with scleroderma and affect a person's ability to open their mouth.

Scientists are continuing to look for a treatment for scleroderma and are optimistic they will find a solution.

The complications of scleroderma range in severity from mild to life threatening. There is also an increased risk of cancer.

Other potential complications include:

Problems with movement and flexibility: Movement may become restricted as the skin tightens and swelling occurs in the hands and fingers, and around the face and mouth. Joint and muscle movement may also become harder.

Raynaud's disease: This disorder can permanently damage the fingertips and toes, resulting in pits or ulcers in the flesh, and possibly gangrene if severe. Amputation may be necessary.

Lung complications: High blood pressure in the artery that carries blood from the heart to the lungs, also called pulmonary hypertension, can cause permanent lung damage and breathing problems. There may be a failure of the right ventricle of the heart. A lung transplant may be necessary.

Kidney damage: This can cause hypertension or high blood pressure, as well as excess protein in the urine. Kidney failure is possible. Symptoms include headache, vision problems, seizures, breathlessness, swelling of the legs and feet, and reduced urine production.

Heart arrhythmias: Abnormal heartbeats and congestive heart failure may result from scarring of heart tissue. A person may develop inflammation of the lining around the heart, known as pericarditis. This causes chest pain and fluid buildup around the heart.

Dental problems: If a tightening of facial skin makes the mouth smaller, even daily dental care can become more difficult. Dry mouth is common, increasing the risk of tooth decay. Acid reflux may destroy tooth enamel, and changes in gum tissue may cause teeth to become loose and fall out.

Sexual dysfunction: Male erectile dysfunction is common in people with scleroderma. A woman's vaginal opening may also constrict, and there can be decreased sexual lubrication.

Hypothyroidism: Scleroderma can cause an underactive thyroid, which may lead to hormonal changes that slow down the metabolism.

Digestive issues: The esophagus may have problems moving solids and liquids into the stomach, resulting in bloating, constipation, and other problems

Most fatalities due to scleroderma relate to lung, heart, and kidney problems.

Living with scleroderma can be challenging, depending on the type and the extent of the impact. There is currently no treatment.

However, there are strategies to reduce some of the negative impacts, including:

eating smaller meals to reduce the impact of GERD

keeping skin well-moisturized and taking care to avoid finger injuries

wrapping up warm to minimize circulatory problems

doing appropriate exercise to ease stiffness

Other tips to manage symptoms include:

avoiding tobacco, alcohol, caffeine, and recreational drugs

getting enough sleep

minimizing stress and managing anxiety, for example, through yoga and meditation

staying well-hydrated

Doctors sometimes advise people with autoimmune conditions such as scleroderma to take Echinacea as it boosts the immune system, but it can be harmful and medical professionals require more research.

There may be a psychological impact, especially if physical changes occur. The support of family and friends can be important to a person with scleroderma.

The Scleroderma Foundation can help a person find advice and support.

The Scleroderma Education Project also provides information about research and advances relating to scleroderma.

Here are a few common questions about scleroderma.

What is the main cause of scleroderma?

Doctors do not know the main causes of scleroderma. However, most believe it to be an autoimmune condition that makes the body produce too much connective tissue.

Is scleroderma a serious condition?

Scleroderma can range in severity from mild to serious. Some types may relate to severe complications that can be life threatening, including heart, kidney, and lung problems.

What does scleroderma do to a person?

Some common early signs of scleroderma include pain, stiffness, swelling, and tightness in the fingers, hands, or feet. This may be more common in the morning or in relation to cold weather or emotional stress.

What is the life expectancy of a person with scleroderma?

The outlook for scleroderma depends on the type and severity of the condition. However, in many cases, scleroderma does not impact overall life expectancy. A 2019 study found that the overall survival rates at 1, 3, 5, and 10 years post-diagnosis were 98.0%, 92.5%, 85.9%, and 71.7%.

Scleroderma refers to several disorders that affect the connective tissues in the body and the skin. The impact on the body can range from mild to severe, depending on the bodily systems it affects.

It is not contagious. It is not clear what exactly causes scleroderma, but certain genetic and environmental factors possibly contribute.

Having a close family member with scleroderma can also significantly increase a person's risk of developing it.

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Conditions of Use and Important Information: This information is meant to supplement, not replace advice from your doctor or healthcare provider and is not meant to cover all possible uses, precautions, interactions or adverse effects. This information may not fit your specific health circumstances. Never delay or disregard seeking professional medical advice from your doctor or other qualified health care provider because of something you have read on WebMD. You should always speak with your doctor or health care professional before you start, stop, or change any prescribed part of your health care plan or treatment and to determine what course of therapy is right for you.

This copyrighted material is provided by Natural Medicines Comprehensive Database Consumer Version. Information from this source is evidence-based and objective, and without commercial influence. For professional medical information on natural medicines, see Natural Medicines Comprehensive Database Professional Version.

© Therapeutic Research Faculty 2018.

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