Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentA Word From Verywell
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
A Word From Verywell
Scleroderma is a chronic,autoimmuneconnective tissue disease that causes changes in the skin, blood vessels, and internal organs due to excess collagen production. While there are different types of scleroderma, each with its own set of symptoms, the most visible manifestation of this disease is skin hardening and tightening. Organs, such as the lungs, kidneys, heart, and digestive tract, may also be affected. There is no cure, but a combination of self-care strategies and medications can help ease symptoms and prevent complications.
Reproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
Scleroderma Symptoms
There are two main types of scleroderma. Localized scleroderma is limited to skin involvement, while systemic sclerosis involves affects blood vessels and internal organs, in addition to the skin. This article will focus mostly on systemic sclerosis (also known simply as scleroderma).
Localized Scleroderma
Localized scleroderma primarily affects only the skin and is seen mostly in children.
Symptoms may include:
Systemic Sclerosis
Systemic sclerosis is divided into two subtypes: limited cutaneous and diffuse cutaneous.
Limited Cutaneous Systemic Sclerosis
In limited cutaneous systemic sclerosis, skin thickening/tightening is usually restricted to certain areas of the body, mostly the hands and face. A subtype of limited cutaneous systemic sclerosis calledCREST syndromeis based on the characteristic symptoms that give it its name.
Symptoms of CREST syndrome:
Diffuse Cutaneous Systemic Sclerosis
Diffuse cutaneous systemic sclerosis causes more extensive skin thickening and tightening, often involving the trunk and moving beyond the hands to above the wrists. Internal organs, such as the lungs, kidneys, heart, and digestive and musculoskeletal systems, are also commonly affected.
For example, joint and muscle pain and swelling of the hands are common in the early stages of scleroderma.
When scleroderma affects the kidneys, a rare but severe complication, calledscleroderma renal crisis, may develop. This causes malignanthigh blood pressurealong with kidney failure.
Scleroderma-related heart problems may manifest asabnormal heart rhythmsorcongestive heart failure.
Interstitial lung disease (scarring of the lung tissue) is more common with diffuse scleroderma, andpulmonary arterial hypertension(high blood pressure in the arteries of the lung) is more common in the limited form. Lung complications are the leading causes of death in systemic sclerosis.
Scleroderma results from abnormalities within three different systems or tissues of the body:
What causes these abnormalities remains largely unknown. Experts suspect that a combination of genetics and exposure to environmental factors could be involved. Environmental exposures that have been evaluated include certain toxins (e.g., polyvinyl chloride, benzene, and silica) or infections with a virus or parasite.
Women between 30 and 50 years old account for 75% of systemic sclerosis cases, but men and children of any age group can also develop it. The condition usually develops between the ages of 25 to 55.
No single test serves as a definitive diagnosis for scleroderma. A combination of findings from a medical history, physical examination, and various studies are used to diagnose this disease.
History and Physical Examination
You should discuss any symptoms with your healthcare provider. For example, you might notice a change in the physical appearance of your face, which could be due to skin hardening and tightening. Or you might be experiencing digestive problems, like acid reflux and swallowing.
Keep in mind, though, that there are other causes besides scleroderma, which will need to be considered. Raynaud’s phenomenon may also occur on its own, meaning it’s not associated withanyunderlying disease process.
Your healthcare provider may refer you to arheumatologist, which is a physician who specializes in the diagnosis and treatment of connective tissue diseases.
Blood Tests
Various blood tests can support a diagnosis of scleroderma.
The vast majority of people with scleroderma are positive for theanti-nuclear antibody (ANA). In addition, the anti-topoisomerase I (anti-Scl-70) antibody is associated with diffuse cutaneous systemic sclerosis; the anticentromere antibody (ACA) is associated with limited cutaneous systemic sclerosis.
Because scleroderma may affect your kidney function, your healthcare provider may also order urine tests and a blood test called thebasic metabolic panel (BMP).
Imaging and Other Tests
Imaging and other tests are used to evaluate for internal organ involvement in scleroderma:
Examples of these tests include:
There is no cure for scleroderma. In other words, there is no medication that can stop or reverse the skin from hardening and thickening. However, through a combination of self-care strategies and medications, many symptoms of scleroderma can be managed and some complications can be prevented.
Here are some examples of how various symptoms/complications are treated in scleroderma.
Raynaud’s Phenomenon
Medications, such ascalcium channel blockersor phosphodiesterase type 5 (PDE-5) inhibitors, may also be used.
Digestive Problems
Along with lifestyle and diet changes, medications to control acid reflux, like the proton pump inhibitor Prilosec (omeprazole), can be helpful.
For problems swallowing related to scleroderma, the drugReglan (metoclopramide)can provide some relief.
Side Effects of Proton Pump Inhibitors
Kidney Disease
Angiotensin-converting enzyme (ACE) inhibitors are medications used to treat kidney problems related to scleroderma. Captopril, a short-acting ACE inhibitor, is considered first-line for treating renal crisis.
Lung Disease
The medicationsCytoxan (cyclophosphamide)or CellCept (mycophenolate mofetil) are used to treat interstitial lung disease.
For people with symptomatic pulmonary arterial hypertension, a variety of medications may be given including:
How to Manage Progressive Pulmonary Hypertension
Muscle and Joint Problems
Muscle and joint pain from scleroderma may be treated with a combination of physical and occupational therapy and medication, like anonsteroidal anti-inflammatory drug (NSAID).
And they are also treated with disease-modifying anti-rheumatic drugs (DMARDs), like hydroxychloroquine, and methotrexate. Steroids are typically avoided because they can precipitate a renal crisis, especially in the diffuse form.
Coping
Living with a chronic, complex, disease like scleroderma is challenging.
In addition to debilitating physical symptoms, people with scleroderma commonly report problems with:
For support, theScleroderma Foundationprovides contact information for individuals and groups throughout the United States you can talk or meet with. Getting to know others who experience your same struggles can be immensely comforting and helpful.
It’s also sensible to work with a therapist, specifically someone who has experience helping people with chronic illnesses.
To optimize your daily functioning and sense of well-being, it’s important to adopt healthy lifestyle habits.
A few examples of these habits include:
If you or a loved one has scleroderma, be sure to seek out care from a team of specialists who have experience treating this uncommon condition and its various manifestations. While scleroderma is a disease that poses unique challenges, there are therapies and strategies out there to help you manage it and live well.
5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American College of Rheumatology.Scleroderma.Kreuter A.Localized scleroderma. Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xAdigun R, Hariz A.Systemic Sclerosis (CREST syndrome)[Updated 2019 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.Sobolewski P, Maślińska M, Wieczorek M, et al.Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619Salazar GA, Assassi S, Wigley F, et al.Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum. 2015;44(6):680-6. doi:10.1016/j.semarthrit.2014.11.006Additional ReadingAdnan ZA.Diagnosis and treatment of scleroderma.Acta Med Indones. Apr;40(2):109-12.Hinchcliff M, Varga J.Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease.Am Fam Physician. 15;78(8):961-68.National Institute of Health: Genetic and Rare Diseases Information Center.Systemic sclerosis.Pattanaik D, Brown M, Postlethwaite BC, Postlethwait AE.Pathogenesis of Systemic Sclerosis.Front Immunol.2015;6:272. doi:10.3389/fimmu.2015.00272
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American College of Rheumatology.Scleroderma.Kreuter A.Localized scleroderma. Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xAdigun R, Hariz A.Systemic Sclerosis (CREST syndrome)[Updated 2019 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.Sobolewski P, Maślińska M, Wieczorek M, et al.Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619Salazar GA, Assassi S, Wigley F, et al.Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum. 2015;44(6):680-6. doi:10.1016/j.semarthrit.2014.11.006Additional ReadingAdnan ZA.Diagnosis and treatment of scleroderma.Acta Med Indones. Apr;40(2):109-12.Hinchcliff M, Varga J.Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease.Am Fam Physician. 15;78(8):961-68.National Institute of Health: Genetic and Rare Diseases Information Center.Systemic sclerosis.Pattanaik D, Brown M, Postlethwaite BC, Postlethwait AE.Pathogenesis of Systemic Sclerosis.Front Immunol.2015;6:272. doi:10.3389/fimmu.2015.00272
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American College of Rheumatology.Scleroderma.Kreuter A.Localized scleroderma. Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xAdigun R, Hariz A.Systemic Sclerosis (CREST syndrome)[Updated 2019 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.Sobolewski P, Maślińska M, Wieczorek M, et al.Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619Salazar GA, Assassi S, Wigley F, et al.Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum. 2015;44(6):680-6. doi:10.1016/j.semarthrit.2014.11.006
American College of Rheumatology.Scleroderma.
Kreuter A.Localized scleroderma. Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.x
Adigun R, Hariz A.Systemic Sclerosis (CREST syndrome)[Updated 2019 Jan 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.
Sobolewski P, Maślińska M, Wieczorek M, et al.Systemic sclerosis - multidisciplinary disease: clinical features and treatment. Reumatologia. 2019;57(4):221-233. doi:10.5114/reum.2019.87619
Salazar GA, Assassi S, Wigley F, et al.Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum. 2015;44(6):680-6. doi:10.1016/j.semarthrit.2014.11.006
Adnan ZA.Diagnosis and treatment of scleroderma.Acta Med Indones. Apr;40(2):109-12.Hinchcliff M, Varga J.Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease.Am Fam Physician. 15;78(8):961-68.National Institute of Health: Genetic and Rare Diseases Information Center.Systemic sclerosis.Pattanaik D, Brown M, Postlethwaite BC, Postlethwait AE.Pathogenesis of Systemic Sclerosis.Front Immunol.2015;6:272. doi:10.3389/fimmu.2015.00272
Adnan ZA.Diagnosis and treatment of scleroderma.Acta Med Indones. Apr;40(2):109-12.
Hinchcliff M, Varga J.Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease.Am Fam Physician. 15;78(8):961-68.
National Institute of Health: Genetic and Rare Diseases Information Center.Systemic sclerosis.
Pattanaik D, Brown M, Postlethwaite BC, Postlethwait AE.Pathogenesis of Systemic Sclerosis.Front Immunol.2015;6:272. doi:10.3389/fimmu.2015.00272
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