What Is Scleroderma?

What Is Scleroderma?
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Scleroderma is a rare autoimmune condition in which the regulation and deposition of collagen is impaired, causing inflammation and fibrosis (thickening and scarring of connective tissue) throughout the body. This includes the blood vessels, internal organs, and digestive tract, and it can have potentially life-threatening consequences.

Unfortunately, scleroderma is a chronic condition for which there is no cure, but with the right treatment and lifestyle choices, you can minimize the impact of scleroderma on your life.

Types of Scleroderma

There are two broad categories of scleroderma: localized and systemic. Localized scleroderma affects only the skin and the area directly underneath, while the effects of systemic scleroderma can spread to tissue, blood vessels, and organs, including those of the respiratory and digestive systems. Each broad category also has two subtypes.

Localized Scleroderma

  • Morphea involves patches of localized scleroderma a half-inch or more in diameter.
  • Linear scleroderma is thickening and forms a line, which usually extends down a limb but can also run down the face.

Systemic Scleroderma

  • Limited cutaneous scleroderma affects the skin slowly over time. It’s typically confined to your fingers, hands, lower arms and legs, usually below the knees.
  • Diffuse cutaneous scleroderma takes effect quickly. It may begin with your fingers and toes before spreading across your arms, legs, and torso and can affect multiple organs, including the gastrointestinal tract, kidneys, lungs, and heart.

Signs and Symptoms of Scleroderma

Scleroderma affects different people in different ways, depending on which kind they have.

 Systemic scleroderma may include some or all of the symptoms of localized scleroderma.

Localized Scleroderma Symptoms

  • Patches of thick, hardened, oval-shaped skin, possibly with a yellow, waxy appearance with an edge resembling a bruise (morphea)
  • Thickened or discolored skin that forms a line running down an arm, leg, and, rarely, forehead

symptoms of scleroderma
Scleroderma can cause any or all of these symptoms in a person with the condition.Everyday Health

Systemic Scleroderma Symptoms

  • Red spots (called telangiectasia)
  • Calcium deposits under the skin, especially the fingertips
  • Raynaud’s phenomenon, which causes pain or numbness in your fingers and toes as a result of contraction of the small blood vessels

  • Bloating
  • Diarrhea
  • Incontinence
  • Constipation
  • Heartburn
  • Difficulty swallowing
  • Shortness of breath
  • Dizziness
  • Fatigue
  • Pulmonary hypertension
  • Heart failure

  • Organ damage
  • Joint pain

Causes and Risk Factors of Scleroderma

Scleroderma’s development is complex and not entirely understood, but dysregulation of the immune system, vascular damage, and excessive deposition of fibrosis and collagen in tissue are thought to play a part. Relatedly, the root cause of scleroderma is unknown, as with many autoimmune disorders, but researchers have identified several possible contributing factors.

  • Genetics There is no single gene for scleroderma, but your genes influence your odds of developing it, the disease’s patterns, and what antibodies associated with the disease are present. People who have a first-degree relative (a parent or sibling) with scleroderma are at higher risk than other people.

  • Environment Certain chemicals or environmental factors may trigger scleroderma, including silica and organic solvents.

  • Gender As many as 80 percent of people diagnosed with scleroderma are women.

     This may be due to hormonal or immunological differences between the sexes.

  • Race People of European descent are more susceptible to localized forms of scleroderma than African Americans, while African Americans and Choctaw Native Americans are at greater risk for systemic scleroderma.

  • Age People between ages 30 and 50 are most susceptible to scleroderma.

How Is Scleroderma Diagnosed?

There is no specific test for scleroderma. The symptoms can differ widely from one person to the next and are similar to those of other autoimmune disorders, so diagnosis is largely about ruling out other conditions. Your doctor may perform a physical exam and ask about your medical history and current and past symptoms. You might also undergo additional tests, including blood tests to assess your immune system and a skin biopsy.

Tests for organ involvement in systemic scleroderma may include:

  • Computerized tomography
  • An echocardiogram
  • Pulmonary function tests

  • A chest X-ray
  • An endoscopy

  • Your primary care physician will also refer you to a rheumatologist for further examination.

Treatment and Medication Options for Scleroderma

Scleroderma has no cure, but the right interventions can help keep your symptoms in check and improve your quality of life.

Medications

  • Anti-inflammatory medications
  • Topical creams or lotions
  • Immunosuppressants
  • Vasodilators, for widening blood vessels

Surgery

Scleroderma does not typically require surgery. But people with severe systemic symptoms may require a stem cell transplant to replace damaged blood cells with healthy cells.

 Complications from scleroderma, such as fibrosis of heart tissue, may also be treated with surgery.

Complementary and Integrative Therapies

  • Physical therapy
  • Light therapy, which uses bright, focused ultraviolet light to treat thickened skin

Lifestyle Changes for Scleroderma

There is no way of preventing scleroderma, but it can be managed with medical treatments and lifestyle changes.

There are a variety of lifestyle changes you may want to consider, depending on your symptoms. The National Institute of Arthritis and Musculosketal and Skin Diseases recommends the following:
  • Stay warm by dressing in layers and wearing gloves and socks.
  • Avoid cold or wet environments that might trigger the symptoms of Raynaud’s phenomenon.
  • Avoid smoking, which causes blood vessels to contract and can worsen your symptoms.
  • Use a humidifier if you’re in a colder climate.
  • Avoid bathing with hot water, which dries out the ski.
  • Get regular exercise, but don’t overdo it. Swimming is particularly beneficial for stimulating blood circulation.
  • See a therapist for help coping with your illness.
  • Visit the dentist regularly to manage effects of the thickening of mouth and facial tissue.
  • For digestive symptoms, eat small, frequent meals, stay hydrated, and reduce or eliminate your intake of alcohol and caffeine.
  • Consider using a wedge pillow or raising the head of your bed with blocks to help.

Scleroderma Prognosis

Scleroderma is a chronic condition with no cure, so you will most likely have symptoms throughout your life.

 As with other autoimmune diseases, you may go through periods in which your symptoms improve or even go into remission, and others in which they flare up or worsen. By making the right lifestyle changes and finding the right treatments, you can reduce the impact scleroderma has on your life.

Complications of Scleroderma

According to Cleveland Clinic, people with scleroderma have a much higher likelihood than the general population to have two conditions: Raynaud’s phenomenon and Sjögren’s syndrome.

As described previously, Raynaud’s phenomenon affects the small blood vessels in your fingers and toes. Specifically, it causes them to contract suddenly, which can cause tingling and numbness and give your digits a pale or bluish appearance. Sjögren’s syndrome is a condition in which certain glands, such as the salivary and tear glands, produce less moisture.

Reduced saliva production can also make you more prone to tooth decay.

Systemic scleroderma can cause a wide range of complications since it can afflict so many parts of the body. Possible complications of systemic forms of scleroderma include:

  • Scarring of heart or lung tissue
  • Cardiovascular issues
  • Inflammation of the sac surrounding the heart
  • A weakened immune system
  • Gastrointestinal diseases
  • Difficulty brushing or cleaning teeth (due to tightening of the facial skin)

Research and Statistics: Who Has Scleroderma?

Scleroderma is rare. An estimated 300,000 Americans have the condition, and within that population, about 100,000 have systemic scleroderma. It’s possible that many people have undiagnosed or misdiagnosed scleroderma given the difficulty of diagnosis.

Anyone can develop scleroderma at any age, but onset is most frequent in adults. Children are more likely to be diagnosed with localized forms of scleroderma, while adults are more likely to have systemic forms of the disease.

Disparities in Scleroderma

Women are far likelier than men to be diagnosed with scleroderma, making up 80 percent of cases.

African Americans are more likely to have systemic scleroderma than non–African Americans. Among African Americans, an estimated 23.7 people per million are diagnosed with systemic scleroderma, compared with 18.3 people per million in non–African Americans.

 Choctaw Native Americans are also more likely than people of European descent to have systemic scleroderma.

Support for Scleroderma

The National Scleroderma Foundation has a network of chapters that provides support and education for people affected by scleroderma in all 50 states, as well as Puerto Rico.

 It also offers access to more than 100 affiliated support groups across the country, as well as virtual groups, led by trained volunteers, that offer support to adults with scleroderma and parents of children with scleroderma.

The Takeaway

  • Scleroderma is an autoimmune condition characterized by impaired regulation and deposition of collagen, which causes inflammation and fibrosis throughout the body, including skin, blood vessels, internal organs, and the digestive tract.
  • Scleroderma can affect anyone of any age, but it is most common in women and adults between 25 and 55.
  • There is no cure for scleroderma, but the right treatments and lifestyle can alleviate your symptoms and minimize their impact on your daily life.

Common Questions & Answers

How do you get scleroderma?
It’s not entirely clear, though researchers believe scleroderma, while not directly inherited from your parents, has a strong genetic component. Certain chemicals and environmental factors, like silica and organic solvents, may trigger the onset of disease.
There is no cure for scleroderma since the cause is not known, but there are treatments and lifestyle changes that can minimize the impact of your symptoms.
Scleroderma is a difficult disease to diagnose since its symptoms overlap with other autoimmune conditions. It is usually diagnosed by a rheumatologist.
Scleroderma varies widely on a case-by-case basis. Localized scleroderma is confined to the skin and the area just underneath, but systemic scleroderma can have effects throughout the body, including the blood vessels, tissues, and organs. Complications from systemic scleroderma can be life-threatening.

Resources We Trust

EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
  1. Scleroderma. Mayo Clinic. June 15, 2024.
  2. Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases. September 2023.
  3. Scleroderma. Cleveland Clinic. September 29, 2023.
  4. Ouchene L et al. Toward Understanding of Environmental Risk Factors in Systemic Sclerosis. Journal of Cutaneous Medicine and Surgery. September 28, 2020.
  5. Scleroderma Risk Factors. Johns Hopkins Medicine.
  6. Scleroderma: Diagnosis, Treatment, and Steps to Take. National Institute of Arthritis and Musculoskeletal and Skin Diseases. September 2023.
  7. Moghaddam MZ et al. Stem cell-based therapy for systemic sclerosis. Rheumatology Advances in Practice. November 20, 2023.
  8. Scleroderma Treatment. Johns Hopkins Medicine.
  9. Who Gets Scleroderma? National Scleroderma Foundation.
  10. Find Your Local Chapter. National Scleroderma Foundation.
  11. Scleroderma Support Groups. National Scleroderma Foundation.
beth-biggee-bio

Beth Biggee, MD

Medical Reviewer

Beth Biggee, MD, is medical director and an integrative rheumatologist at Rheumission, a virtual integrative rheumatology practice for people residing in California and Pennsylvania. This first-of-its-kind company offers whole person autoimmune care by a team of integrative rheumatologists, lifestyle medicine practitioners, autoimmune dietitians, psychologists, and care coordinators.

Dr. Biggee also works as a healthcare wellness consultant for Synergy Wellness Center in Hudson, Massachusetts. Teamed with Synergy, she provides in-person lifestyle medicine and holistic consults, and contributes to employee workplace wellness programs. She has over 20 years of experience in rheumatology and holds board certifications in rheumatology and integrative and lifestyle medicine. Dr. Biggee brings a human-centered approach to wellness rather than focusing solely on diseases.

Dr. Biggee graduated cum laude with a bachelor's degree from Canisius College, and graduated magna cum laude and as valedictorian from SUNY Health Science Center at Syracuse Medical School. She completed her internship and residency in internal medicine at Yale New Haven Hospital, completed her fellowship in rheumatology at Tufts–New England Medical Center, and completed training in integrative rheumatology at the University of Arizona Andrew Weil Center for Integrative Medicine. Following her training, she attained board certification in rheumatology and internal medicine through the American Board of Internal Medicine, attained board certification in integrative medicine through the American Board of Physician Specialties, and attained accreditation as a certified lifestyle medicine physician through the American College of Lifestyle Medicine. She is certified in Helms auricular acupuncture and is currently completing coursework for the Aloha Ayurveda integrative medicine course for physicians.

In prior roles, Dr. Biggee taught as an assistant clinical professor of medicine at Mary Imogene Bassett Hospital (an affiliate of Columbia University). She was also clinical associate of medicine at Tufts University School of Medicine and instructed "introduction to clinical medicine" for medical students at Tufts. She was preceptor for the Lawrence General Hospital Family Medicine Residency.

Dr. Biggee has published in Annals of Rheumatic Diseases, Arthritis in Rheumatism, Current Opinions in Rheumatology, Journal for Musculoskeletal Medicine, Medicine and Health Rhode Island, and Field Guide to Internal Medicine.

Ajai Raj

Author
Ajai Raj is a reporter specializing in medical science, health, and technology. His work has appeared in Popular Science, Scientific American Mind, The Scientist, and the New York Daily News, as well as a number of medical trade journals.