Table of ContentsView AllTable of ContentsSymptoms of Localized SclerodermaSymptoms of Systemic SclerosisRare SymptomsComplicationsPrognosis
Table of ContentsView All
View All
Table of Contents
Symptoms of Localized Scleroderma
Symptoms of Systemic Sclerosis
Rare Symptoms
Complications
Prognosis
Sclerodermais a chronic autoimmune disease of the connective tissue that causes a hardening and tightening of the skin. (Scleroderma means “hard skin” in Greek). The disease can be localized, with symptoms related entirely to the skin, or it can be systemic, in which hardening can occur in connective tissues within the body.
Nearly everyone with scleroderma develops patches of thick, hardened skin. Other common symptoms include muscle and joint pain, gastrointestinal (GI) issues, swollen hands, narrowing of blood vessels,calcium depositsunder the skin, and more.
This article discusses the two main forms of scleroderma and their symptoms. It also includes scleroderma pictures and covers potential complications of the condition.
Verywell / Emily Roberts
Types of Scleroderma
The two main categories of scleroderma include:
Localized scleroderma affects mostly children and is less severe than systemic sclerosis, which is more common in adults. The causes of scleroderma are still unknown, but it is believed to be related to a buildup of collagen (a protein in connective tissue) in the skin and an abnormal immune system response.
Localized scleroderma may appear in one spot or in several patches or regions of the skin. It has the following two main subtypes.
What Triggers a Scleroderma Flare-Up?It’s not clear what exactly causes scleroderma, however, certain environmental triggers have been linked to the condition. These potential triggers include exposure to silica and certain solvents and drugs.
What Triggers a Scleroderma Flare-Up?
It’s not clear what exactly causes scleroderma, however, certain environmental triggers have been linked to the condition. These potential triggers include exposure to silica and certain solvents and drugs.
Common Symptoms of Localized Scleroderma
Localized scleroderma is a rare condition. Linear scleroderma typically involves both the surface and deeper layers of the skin, but morphea scleroderma doesn’t tend to go beyond the surface layers of theepidermisanddermis.
Linear Scleroderma
Linear scleroderma forms into lines as it hardens. It often occurs on one side of the body and can appear as a line down an arm or leg, or sometimes the head. As it spreads to deeper skin layers, it may also involve muscle and bone. Linear scleroderma typically occurs in children.
Common symptoms of linear scleroderma include:
Morphea Scleroderma
Morpheascleroderma, the more common form of localized scleroderma, most often forms on the abdomen and back. It can also sometimes develop on the face, arms, and legs.
Morphea scleroderma can be limited to one to four small patches (plaque morphea) or in some cases it can spread over large areas of the body (generalized morphea). It most commonly occurs in adults between the ages of 20 and 50, but can also occur in children.
Common symptoms include:
Morphea scleroderma tends to be oval and reddish, but the waxy patches may vary in color, shade (light or dark), size, and shape. The patches may get larger or shrink, and they may disappear spontaneously.
Reproduced with permission from ©DermNet New Zealand and © Raimo Suhonenwww.dermnetnz.org2023.
Localized scleroderma typically goes away over time, but it may leave skin discoloration even after it resolves. Linear scleroderma typically lasts two to five years, and morphea lesions last an average of three to five years.
Common Symptoms of Systemic Sclerosis
Systemic sclerosis (SSc) can affect connective tissues in many parts of the body. The two main subtypes are limited cutaneous SSc, which progresses slowly over a period of years, and diffuse cutaneous SSc, which covers more skin area and progresses more quickly to multiple organs and systems.
Limited Cutaneous Systemic Sclerosis
Limited cutaneous SSc is also calledCREST syndrome, an acronym for its common conditions and symptoms.
CREST syndromePeople with limited cutaneous SSc may have two or more common features of CREST syndrome:Calcinosis,which arecalcium deposits in the skinRaynaud’s phenomenon,a spasm of blood vessels in response to cold, stress, or emotional upset that can decrease blood flow in fingers or toesEsophageal dysfunction,which is when smooth muscles that line the esophagus lose normal movement and functionSclerodactyly,a thickening and tightening of the skin of the fingers and handsTelangiectasia, a swelling of capillaries (small blood vessels) near the surface of the skin on the face and hands that causes red spots
CREST syndrome
People with limited cutaneous SSc may have two or more common features of CREST syndrome:Calcinosis,which arecalcium deposits in the skinRaynaud’s phenomenon,a spasm of blood vessels in response to cold, stress, or emotional upset that can decrease blood flow in fingers or toesEsophageal dysfunction,which is when smooth muscles that line the esophagus lose normal movement and functionSclerodactyly,a thickening and tightening of the skin of the fingers and handsTelangiectasia, a swelling of capillaries (small blood vessels) near the surface of the skin on the face and hands that causes red spots
People with limited cutaneous SSc may have two or more common features of CREST syndrome:
Common skin-related symptoms of limited cutaneous SSc include:
An estimated 95% of SSc cases begin with Raynaud’s phenomenon. In limited cutaneous SSc, Raynaud’s phenomenon often occurs for several years prior to skin thickening.
Early symptoms of limited cutaneous SSc involve sensitivity and/or swelling of fingers or toes. The swelling of the hands may be especially pronounced in the morning due to muscle inactivity during sleeping hours. Fingers may look sausage-like, making it difficult to close the hand into a fist. Symptoms may subside as the day goes on.
Reproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
Gastrointestinal issues affect up to 90% of people who have SSc. The esophagus is the most commonly involved organ, affecting 67% of people with SSc.The symptoms are due to structural and functional changes of the esophagus that can occur in limited or diffuse cutaneous SSc.
Common esophageal symptoms include:
Heartburn Relief: Natural Antacids, Medication, Prevention
Sometimes excess collagen collects in the tissue between the lungs' air sacs in people with limited cutaneous SSc, making the lung tissue stiffer and less able to work properly. If the lungs become affected, common additional symptoms include:
Diffuse Cutaneous Systemic Sclerosis
Raynaud’s phenomenon often occurs simultaneously or just prior to skin thickening in those with diffuse cutaneous SSc. Diffuse cutaneous SSc can involve the heart, lungs, kidneys, gastrointestinal tract, and central and peripheral nervous systems.
Common symptoms of diffuse cutaneous SSc include:
Muscle and joint pain may also occur along tendons and in muscles in the arms and legs. This can worsen with movement of the ankles, wrists, knees, or elbows. As the disease progresses, muscle loss and weakness may develop along with swelling, warmth, and tenderness around the joints and muscles.
Often, people with diffuse cutaneous SSc experience a grating noise when they try to move inflamed joints, particularly joints at and below the knees.
In the lower GI tract, diffuse cutaneous SSc can sometimes slow movement of food and reduce food absorption.
In both types of systemic sclerosis, lumps of calcinosis may break through the skin and leak a white substance. The open cuts may then become infected.
There are rare subtypes of localized scleroderma that can sometimes develop into more serious conditions:
Some people with SSc experience additional symptoms that may or may not seem related to the disease. This can include:
Scleroderma can cause many complications that range from mild to life-threatening. These usually occur when the disorder has not been treated or when treatment has failed to adequately address symptoms.
Complications of Localized Scleroderma
Complications of localized scleroderma may include the following.
Complications of Systemic Sclerosis
Complications of SSc are most often related to diffuse cutaneous SSc and can involve many organs and body systems. They may include the following.
Outlook for Scleroderma
The outlook for scleroderma can vary widely from person to person. However, many people with scleroderma—particularly those with limited disease—go on to live normal lifespans.
While there is no cure for scleroderma, there are treatments available that can help relieve symptoms and prevent complications. Treatment is most effective when started early.
Localized scleroderma typically gets better on its own; there will likely come a time when no new lesions form and the existing ones fade. Systemic sclerosis is manageable with treatments to target affected organs. These treatments are continuously improving and can help with symptoms and improve quality of life.
When to See a Healthcare Provider
Individuals who exhibit symptoms of scleroderma should seek out a physician with expertise in this complex disease, such as a rheumatologist. Early detection of scleroderma can help reduce the risk of serious complications.By recognizing and treating organ involvement in SSc early, you can prevent irreversible damage.
Summary
Scleroderma is a chronic autoimmune disease that causes inflammation and the build up of fibrous tissue in the skin and other areas of the body. The disease may be localized or systemic (affecting the whole body). Symptoms of scleroderma include thickened skin, skin color changes, stiff joints, muscle weakness, visible blood vessels, and more. Although there is no cure for the condition, there are treatments available that can help relieve symptoms and improve your quality of life.
12 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.National Center for Advancing Translational Sciences.Localized scleroderma.Johns Hopkins Medicine.Scleroderma risk factors.Careta MF, Romiti R.Localized scleroderma: clinical spectrum and therapeutic update.An Bras Dermatol.2015;90(1):62-73. doi:10.1590/abd1806-4841.20152890Mount Sinai.Scleroderma.DermNet.Morphoea.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.2019.87619Nawaz I, Nawaz Y, Nawaz E, Manan MR, Mahmood A.Raynaud’s phenomenon: reviewing the pathophysiology and management strategies.Cureus. 2022;14(1):e21681. doi:10.7759/cureus.21681Valenzuela A, Song P, Chung L.Calcinosis in scleroderma.Curr Opin Rheumatol2018;30(6):554-561. doi:10.1097/BOR.0000000000000539National Scleroderma Foundation.Localized scleroderma.Reiff D, Crayne CB, Mannion ML, Cron RQ.Characteristics of coexisting localized scleroderma and inflammatory arthritis.Eur J Rheumatol. 2020;7(1):67-71. doi:10.5152/eurjrheum.2019.19147Parrado RH, Lemus HN, Coral-Alvarado PX, Quintana López G.Gastric antral vascular ectasia in systemic sclerosis: current concepts.International Journal of Rheumatology. 2015;2015:1-6. doi:10.1155/2015/762546American Academy of Dermatology.Scleroderma: diagnosis and treatment.Additional ReadingAmerican College of Rheumatology.Scleroderma.Henderson W. Scleroderma News.7 scleroderma complications.Kreuter A.Localized scleroderma.Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xNational Institute of Arthritis and Musculoskeletal and Skin Diseases.Raynaud’s phenomenon.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Scleroderma.
12 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.National Center for Advancing Translational Sciences.Localized scleroderma.Johns Hopkins Medicine.Scleroderma risk factors.Careta MF, Romiti R.Localized scleroderma: clinical spectrum and therapeutic update.An Bras Dermatol.2015;90(1):62-73. doi:10.1590/abd1806-4841.20152890Mount Sinai.Scleroderma.DermNet.Morphoea.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.2019.87619Nawaz I, Nawaz Y, Nawaz E, Manan MR, Mahmood A.Raynaud’s phenomenon: reviewing the pathophysiology and management strategies.Cureus. 2022;14(1):e21681. doi:10.7759/cureus.21681Valenzuela A, Song P, Chung L.Calcinosis in scleroderma.Curr Opin Rheumatol2018;30(6):554-561. doi:10.1097/BOR.0000000000000539National Scleroderma Foundation.Localized scleroderma.Reiff D, Crayne CB, Mannion ML, Cron RQ.Characteristics of coexisting localized scleroderma and inflammatory arthritis.Eur J Rheumatol. 2020;7(1):67-71. doi:10.5152/eurjrheum.2019.19147Parrado RH, Lemus HN, Coral-Alvarado PX, Quintana López G.Gastric antral vascular ectasia in systemic sclerosis: current concepts.International Journal of Rheumatology. 2015;2015:1-6. doi:10.1155/2015/762546American Academy of Dermatology.Scleroderma: diagnosis and treatment.Additional ReadingAmerican College of Rheumatology.Scleroderma.Henderson W. Scleroderma News.7 scleroderma complications.Kreuter A.Localized scleroderma.Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xNational Institute of Arthritis and Musculoskeletal and Skin Diseases.Raynaud’s phenomenon.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Scleroderma.
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.
National Center for Advancing Translational Sciences.Localized scleroderma.Johns Hopkins Medicine.Scleroderma risk factors.Careta MF, Romiti R.Localized scleroderma: clinical spectrum and therapeutic update.An Bras Dermatol.2015;90(1):62-73. doi:10.1590/abd1806-4841.20152890Mount Sinai.Scleroderma.DermNet.Morphoea.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.2019.87619Nawaz I, Nawaz Y, Nawaz E, Manan MR, Mahmood A.Raynaud’s phenomenon: reviewing the pathophysiology and management strategies.Cureus. 2022;14(1):e21681. doi:10.7759/cureus.21681Valenzuela A, Song P, Chung L.Calcinosis in scleroderma.Curr Opin Rheumatol2018;30(6):554-561. doi:10.1097/BOR.0000000000000539National Scleroderma Foundation.Localized scleroderma.Reiff D, Crayne CB, Mannion ML, Cron RQ.Characteristics of coexisting localized scleroderma and inflammatory arthritis.Eur J Rheumatol. 2020;7(1):67-71. doi:10.5152/eurjrheum.2019.19147Parrado RH, Lemus HN, Coral-Alvarado PX, Quintana López G.Gastric antral vascular ectasia in systemic sclerosis: current concepts.International Journal of Rheumatology. 2015;2015:1-6. doi:10.1155/2015/762546American Academy of Dermatology.Scleroderma: diagnosis and treatment.
National Center for Advancing Translational Sciences.Localized scleroderma.
Johns Hopkins Medicine.Scleroderma risk factors.
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Mount Sinai.Scleroderma.
DermNet.Morphoea.
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
Nawaz I, Nawaz Y, Nawaz E, Manan MR, Mahmood A.Raynaud’s phenomenon: reviewing the pathophysiology and management strategies.Cureus. 2022;14(1):e21681. doi:10.7759/cureus.21681
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National Scleroderma Foundation.Localized scleroderma.
Reiff D, Crayne CB, Mannion ML, Cron RQ.Characteristics of coexisting localized scleroderma and inflammatory arthritis.Eur J Rheumatol. 2020;7(1):67-71. doi:10.5152/eurjrheum.2019.19147
Parrado RH, Lemus HN, Coral-Alvarado PX, Quintana López G.Gastric antral vascular ectasia in systemic sclerosis: current concepts.International Journal of Rheumatology. 2015;2015:1-6. doi:10.1155/2015/762546
American Academy of Dermatology.Scleroderma: diagnosis and treatment.
American College of Rheumatology.Scleroderma.Henderson W. Scleroderma News.7 scleroderma complications.Kreuter A.Localized scleroderma.Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.xNational Institute of Arthritis and Musculoskeletal and Skin Diseases.Raynaud’s phenomenon.National Institute of Arthritis and Musculoskeletal and Skin Diseases.Scleroderma.
American College of Rheumatology.Scleroderma.
Henderson W. Scleroderma News.7 scleroderma complications.
Kreuter A.Localized scleroderma.Dermatol Ther. 2012;25(2):135-47. doi:10.1111/j.1529-8019.2012.01479.x
National Institute of Arthritis and Musculoskeletal and Skin Diseases.Raynaud’s phenomenon.
National Institute of Arthritis and Musculoskeletal and Skin Diseases.Scleroderma.
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