Arthritis More Arthritis Types & Related Conditions Symptoms of Scleroderma By Lana Barhum facebook linkedin Lana Barhum has been a freelance medical writer for over 10 years. She shares advice on living well with chronic disease. Learn about our editorial process Lana Barhum Medically reviewed by Medically reviewed by Casey Gallagher, MD on October 15, 2018 Casey Gallagher, MD, is board-certified in dermatology. He is a clinical professor at the University of Colorado in Denver, and co-founder and practicing dermatologist at the Boulder Valley Center for Dermatology in Colorado. His research has been published in the New England Journal of Medicine. Learn about our Medical Review Board Casey Gallagher, MD Updated on December 12, 2020 Print Table of Contents View All Table of Contents Types Common Symptoms of Localized Scleroderma Common Symptoms of Systemic Scleroderma Rare Symptoms Complications Scleroderma is 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 all related to the skin or it can be systemic and the hardening can occur in connective tissues within the body. Nearly everyone with scleroderma develops patches of thick, hardened skin. The region will depend on the type of scleroderma but it often includes the hands, arms, and/or face, and sometimes the torso and legs. As the skin tightens, movement can become restricted and joint and muscle pain can occur. The systemic form, also called systemic sclerosis (SSc) also commonly causes skin discoloration, swollen hands, narrowing of blood vessels in the hands and feet in response to cold, red spots on the face from swelling blood vessels, calcium deposits under the skin, and heartburn and other gastrointestinal issues. In addition, the lungs, heart, or kidneys can become affected and stop functioning efficiently, which can lead to serious symptoms and complications, such as shortness of breath and irregular heart rhythms (arrhythmias). Treatments vary greatly depending on the type of scleroderma and symptoms, but may include topical and/or oral medications and physical therapy under the care and monitoring of a physician, such as a rheumatologist (a specialist in autoimmune and inflammatory conditions). Verywell / Emily Roberts Types The two main categories of scleroderma are localized scleroderma, which affects the skin and occasionally the underlying muscles or joints, and systemic scleroderma, which involves blood vessels and internal organs throughout the body. Localized scleroderma affects mostly children and is less severe than systemic sclerosis, which is more common in adults. 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 two main subtypes: Linear scleroderma: Lesions look like straight lines or streaksMorphea: Reddish oval patches form A Look at the Different Types of Scleroderma (Systemic Sclerosis) 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 doesn't tend to go beyond the surface layers of the epidermis and dermis. 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: Lines or streaks of hardened, waxy skin on the trunk and limbs or face and scalpSkin discoloration that may appear lighter or darkerJoint tightness Morphea Morphea scleroderma, 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 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). Morphea most commonly occurs in adults between the ages of 20 and 50, but it can also occur in children. Common symptoms include: Patches of reddish skin that thicken into firm, oval-shaped areas on the abdomen, chest, or backSkin becomes waxy and shiny as it tightensCenter of the patches can be ivory or yellow with violet bordersPruritis (itchy skin) Morphea 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. 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 Scleroderma Systemic scleroderma can affect connective tissues in many parts of the body. The two main subtypes are limited scleroderma, which progresses slowly over a period of years, and diffuse scleroderma, which covers more skin area and progresses quickly to multiple organs and systems. Limited Scleroderma Limited scleroderma is also called limited cutaneous systemic sclerosis or CREST syndrome, an acronym for its common conditions and symptoms. CREST People with limited scleroderma may have two or more common features of CREST:Calcinosis, which are calcium 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 Common skin-related symptoms of limited scleroderma/CREST include: Fingers or toes turn blue and/or white when cold and then become bright red when warmed back upSwelling and sausage-like appearance of fingersSkin thickening on the face, arms, and legsSmall, hard lumps in or under the skin (calcinosis)Fingers curl due to skin tighteningRange of finger motion is reducedWaxy, mask-like appearance of faceTiny red spots on hands and faceAbnormal skin dryness An estimated 95% of systemic scleroderma cases begin with Raynaud's phenomenon, according to the American Scleroderma Foundation. In limited scleroderma, Raynaud's phenomenon often occurs for several years prior to skin thickening. Early symptoms of limited scleroderma 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. Gastrointestinal issues affect up to 90% of people who have systemic scleroderma. The esophagus is the most commonly involved organ, affecting 67% of people with systemic scleroderma. The symptoms are due to structural and functional changes of the esophagus that can occur in limited or diffuse scleroderma Common esophageal symptoms include: Heartburn (the sensation of burning behind the breast bone)Difficulty or pain while swallowingRegurgitationHoarsenessMouth ulcersAcid taste in the mouth The most common symptom is heartburn. This is due to irritation of the esophagus by acid reflux (stomach acid backing up or refluxing up the esophagus). How Heartburn Is Treated Sometimes excess collagen collects in the tissue between the lungs' air sacs in people with limited scleroderma, making the lung tissue stiffer and less able to work properly. If the lungs become affected, common additional symptoms include: Persistent coughShortness of breath Diffuse Scleroderma Raynaud's phenomenon often occurs simultaneously or just prior to skin thickening in those with diffuse scleroderma. Diffuse scleroderma can involve the heart, lungs, kidneys, gastrointestinal tract, and central and peripheral nervous systems. Common symptoms of diffuse scleroderma include: Swelling and sausage-like appearance of fingersSkin thickening over large areas of the torso, hands, arms, and legsWaxy, mask-like appearance of faceCRESTSkin darkening or a salt-and-pepper appearance Thinning of lips and furrowing around mouthMuscle and joint pain and/or stiffnessGrating noise with movement of jointsWeight lossFatigueHeartburnGastroparesis (sensation of nausea, fullness, or bloating from retention of food in stomach)Cramps and/or diarrheaChronic coughShortness of breath Muscle and joint pain may also occur along tendons and in muscles of 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 scleroderma experience a grating noise when they try to move inflamed joints, particularly joints at and below the knees. In the lower GI tract, diffuse scleroderma can sometimes slow movement of food and reduce food absorption. In both types of systemic scleroderma, lumps of calcinosis may break through the skin and leak a white substance. The open cuts may then become infected. Rare Symptoms There are rare subtypes of localized scleroderma that can sometimes be more serious conditions. These types include: Subcutaneous morphea affects deeper tissues and can extend deep into muscles.Bullous morphea causes blister-like bumps or areas of erosion on the region of morphea.Generalized morphea may also limit joint function due to its larger coverage areas. In rare cases, the larger lesions can merge together, covering the entire body.En coup de sabre is a rare form of linear scleroderma that affects the head, especially the forehead. Lesions form depressed grooves that resemble a sword wound or the stroke of a sabre. If it's on the scalp, hair loss can occur. In rare cases, en coup de sabre can cause abnormalities in the growth of facial bones and, unlike other forms of limited scleroderma, it can recur many years after going away. Some people with systemic scleroderma experience additional symptoms that may or may not seem related to the disease. This can include: Trigeminal neuralgia (sudden episodes of severe facial pain)Limited eye movementSevere fatigue and depression Complications 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 include: Joint pain: About 10% to 20% of people with localized scleroderma develop pain in affected joints.Slowed growth of an arm or leg: Linear scleroderma that extends to muscles and tendons can sometimes stop or slow the growth of the affected limb in children.Co-existing inflammatory arthritis and juvenile idiopathic arthritis (JIA): When localized scleroderma and arthritis occur together, lesions can cover arthritic joints, and the arthritis may be less responsive or unresponsive to treatments. Complications of Systemic Scleroderma Complications of systemic scleroderma are most often related to diffuse scleroderma and can involve many organs and body systems. Complications can include: Ulcers on fingers and toes: Severe Raynaud's phenomenon can obstruct the blood flow to fingers and toes, causing ulcers that can be difficult to heal. Fingertips may be permanently damaged.Gangrene: In extreme cases, abnormal or narrowed blood vessels combined with severe Raynaud's can lead to gangrene and may necessitate amputation.Incontinence: A weakening of the muscles caused by a decrease in the blood supply to the nerves can lead to gastrointestinal discomfort and urinary or fecal incontinence.Lung damage: Breathing problems due to pulmonary hypertension and scarring of the connective tissue in the lungs can lead to lung disease and potentially heart failure.Heart problems: Scarring of heart tissue and narrowed blood vessels can lead to abnormal heart rhythms and, in rare cases, to an inflamed heart muscle (myocarditis).Kidney damage: Restricted blood flow to the kidneys can result in scleroderma renal crisis. If left untreated, this condition, can lead to malignant high blood pressure and kidney failure.Dental problems: Severe tightening of facial skin can make it difficult to open your mouth wide enough to brush your teeth. Additionally, acid reflux can destroy tooth enamel, and changes in gum tissue due to scleroderma may cause teeth to become loose or fall out.Dry eyes and mouth: Diffuse scleroderma can cause very dry eyes and mouth, a condition known as Sjogren's syndrome.Gastric antral vascular ectasia syndrome (GAVE): Sometimes called "watermelon stomach," this is a rare condition resulting in red-streaked areas in the stomach from widened blood vessels, and iron deficiency anemia. GAVE, which affects an estimated 5% of people with systemic scleroderma, increases the risk of stomach cancer.Sexual dysfunction: Males with scleroderma may experience erectile dysfunction; women may have decreased lubrication and a constricted vaginal opening.Heart failure: Scleroderma-related heart problems may manifest as abnormal heart rhythms or congestive heart failure.Nervous system problems: Headache and dizziness, convulsions, and visual disturbances can occur if the nervous system is affected. In some cases it can also lead to aphasia (loss of ability to speak and understand language). When to See a Doctor 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 systemic scleroderma early, you can prevent irreversible damage. A Word From Verywell If you or your child is diagnosed with scleroderma, it can be overwhelming, but it's important to remember that localized scleroderma typically goes away over time and systemic scleroderma is manageable with treatments to target affected organs. If it's localized scleroderma, there will likely come a time when no new lesions form and the existing ones fade. With systemic scleroderma, treatments are continuously improving and can help you with your symptoms and improve your quality of life. Tips for Living Well With Scleroderma Was this page helpful? Thanks for your feedback! Dealing with chronic inflammation? An anti-inflammatory diet can help. Our free recipe guide shows you the best foods to fight inflammation. Get yours today! Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit Article Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Ferreli C, Gasparini G, Parodi A, et al. Cutaneous manifestations of scleroderma and scleroderma-like disorders: A comprehensive review. Clin Rev Allergy Immunol. 2017;53(3):306-336. doi:10.1007/s12016-017-8625-4 National Center for Advancing Translational Sciences. Scleroderma. Updated September 12, 2016. Careta MF, Romiti R. Localized scleroderma: Clinical spectrum and therapeutic update. An Bras Dermatol. 2015;90(1):62-73. doi:10.1590/abd1806-4841.20152890 Scleroderma Foundation. What is scleroderma? National Center for Advancing Translational Sciences. Morphea. Updated February 15, 2018. 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 Shah AA, Wigley FM. My approach to the treatment of scleroderma. Mayo Clin Proc. 2013;88(4):377-93. doi:10.1016/j.mayocp.2013.01.018 Valenzuela A, Song P, Chung L. Calcinosis in scleroderma. Curr Opin Rheumatol 2018;30(6):554-561. doi:10.1097/BOR.0000000000000539 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 Fuccio L, Mussetto A, Laterza L, Eusebi LH, Bazzoli F. Diagnosis and management of gastric antral vascular ectasia. World J Gastrointest Endosc. 2013;5(1):6-13. doi:10.4253/wjge.v5.i1.6 Additional Reading American College of Rheumatology, Scleroderma Fact Sheet Careta MF, Romiti R. Localized scleroderma: Clinical spectrum and therapeutic update.An Bras Dermatol. 2015;90(1):62-73. doi:10.1590/abd1806-4841.20152890 Henderson W. Scleroderma News. 7 scleroderma complications. Published August 24, 2017. 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. Scleroderma. Last Reviewed August 30, 2016. National Institute of Arthritis and Musculoskeletal and Skin Diseases. What is raynaud’s phenomenon? Updated October 30, 2016. Parrado RH, Lemus HN, Coral-Alvarado PX, et al. Gastric antral vascular ectasia in systemic sclerosis: Current concepts. Int J Rheumatol. 2015;2015:762546.doi:10.1155/2015/762546 Sobolewski P, Maślińska M, Wieczorek M, et al. Systemic sclerosis - Multidisciplinary disease: Clinical Features and Treatment. Reumatologia. 2019;57(4):221-233 doi: