Juvenile Systemic Scleroderma: Understanding Symptoms, Types, and Care
Updated: 3 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick Answer: Juvenile Scleroderma at a Glance
Juvenile systemic scleroderma is a rare autoimmune disease in children that causes tissue scarring [1], with hard or tight skin, Raynaud's phenomenon and joint stiffness [1]. It can affect the lungs, heart, kidneys and digestive tract [1]. Fingers or toes changing color with cold or stress is an early sign [1]. There is no cure [1], but treatment aims to protect organ function.
Feature | Details |
What it is | A rare autoimmune disease in children that causes tissue scarring (fibrosis) [1]. |
How common | About 5,000 to 7,000 children in the United States have scleroderma of any type [1]; the systemic form is rare. |
Primary symptoms | Hard or tight skin, Raynaud's phenomenon, and joint stiffness [1]. |
Internal impact | Can affect the lungs, heart, kidneys, and digestive tract [1]. |
Early warning sign | Fingers or toes turning white, blue, or red with cold or stress [1]. |
Main specialists | Rheumatologists and dermatologists [1]. |
Treatment goals | Reduce inflammation, keep the disease from getting worse, and protect organ function [1]. |
Outlook | No cure; a long-term condition that can stay stable for years [1]. |
What the research says
The disease often involves more than one organ system. In a North American registry of 64 children with juvenile systemic sclerosis (median age at onset 10.3 years), the most common problems were in the skin and blood vessels, followed by the muscles and joints, digestive tract, and lungs, and 38% had four or more organ systems affected. Disability was greater than in children with juvenile arthritis, dermatomyositis, or lupus, and digestive involvement had the greatest effect on quality of life. Over more than 50 person-years of follow-up, most organ problems stayed stable and no deaths were reported. (Stevens et al., Arthritis Care Res, 2018) [2]
The two main subtypes behave differently. In an international cohort of 150 children from 42 centers, 72% had the diffuse cutaneous subtype. Compared with the limited subtype, the diffuse subtype had thicker skin, more finger-tip ulcers, more lung involvement, and worse physician-rated disease activity and damage, while the limited subtype had more heart involvement. (Foeldvari et al., Arthritis Care Res, 2022) [3]
It is rare, and major organ involvement is common. A nationwide survey in Japan estimated about 299 patients with juvenile-onset systemic sclerosis, with an annual incidence of 0.98 to 1.59 per million children from 2016 to 2020. Among 130 cases analyzed, interstitial lung disease was present in 40.8%, digestive tract problems in 36.9%, and pulmonary arterial hypertension in 7.7%, with no kidney crisis. (Kaneko et al., J Rheumatol, 2026) [4]
What Is Juvenile Systemic Scleroderma?
Juvenile systemic scleroderma is a condition in which a child's immune system attacks the body's healthy cells [1]. This creates inflammation in the skin, and the swelling triggers an overproduction of collagen, a protein that supports tissues and helps them stay flexible [1]. Too much collagen causes fibrosis, or scarring, so the skin and affected internal organs become unusually thick and hard [1].
The word "systemic" matters. It means the condition can affect the whole body [1], which sets it apart from localized scleroderma, which affects the skin in only some areas of the body and rarely involves internal organs [1]. Because systemic scleroderma is rare in children, specialized medical knowledge is needed to manage it.
Who Gets It and What Causes It?
Juvenile systemic scleroderma can affect anyone of any age or sex, but it is rare in early childhood [1]. About 5,000 to 7,000 children in the United States have scleroderma of any type [1]. Among all people with scleroderma, only about 2% develop it before age 10 and about 7% between ages 10 and 19 [1].
The exact cause is unknown [1]. Research suggests that an injury to the cells lining the blood vessels over-activates the skin's connective tissue cells (fibroblasts), which make collagen [1]. The buildup of collagen produces the symptoms. Because the cause is unknown, there is no known way to prevent the condition [1].
What types of juvenile systemic scleroderma affect children?
Juvenile systemic scleroderma in children comes in three types, based on how the disease shows up in the body [1].
Diffuse Cutaneous Systemic Sclerosis
Symptoms include widespread hardening of the skin and problems with organ function throughout the body.
Limited Cutaneous Systemic Sclerosis (CREST Syndrome)
CREST is an acronym for the symptoms: Calcinosis (calcium deposits under the skin), Raynaud's phenomenon, Esophageal dysfunction (heartburn), Sclerodactyly (thick skin on the fingers), and Telangiectasia (enlarged blood vessels) [1].
Limited Systemic Sclerosis (Sine Scleroderma)
This form affects organ function but does not cause the skin to thicken [1].
What are the symptoms of juvenile systemic scleroderma?
Symptoms vary depending on which parts of the body are affected. Pain is usually not severe, so a child can have scleroderma without knowing it [1].
Skin and Tissue Changes
At first, the skin of the hands and feet may look swollen [1]. Over time, the skin tightens and hardens, loses its ability to stretch, and may develop ridges, depressed areas, or small pits [1]. Tight skin on the fingers and hands can reduce hand function [1]. Parents may also notice enlarged red blood vessels (telangiectasias) on the hands, face, and around the nail beds [1], or hard calcium lumps (calcinosis) under the skin [1].
Internal Organ Involvement
When internal organs are involved, a child may have:
Joint inflammation with stiffness and pain [1]
Sores (ulcers), mostly on the fingertips [1]
Digestive problems such as heartburn, trouble swallowing, diarrhea, or stomach cramps [1]
Breathing problems such as a chronic cough or difficulty breathing [1]
Kidney problems that lead to high blood pressure [1]
Fatigue and muscle weakness [1]
Why is Raynaud's phenomenon important in juvenile systemic scleroderma?
Raynaud's phenomenon is one of the earliest signs of systemic sclerosis [1]. In response to cold or stress, the fingers and toes change color [1], typically to white, then blue, then red as blood flow returns. Because it appears early in the course of the disease and can be a sign of systemic sclerosis [1], it is an important reason for parents to seek a specialist's opinion.
How is juvenile systemic scleroderma diagnosed?
Juvenile systemic scleroderma is diagnosed from a child's symptoms and physical signs, characteristic skin changes, and a complete medical history and physical exam [1]. The provider often teams up with a rheumatologist, a dermatologist, and specialists for the lungs, heart, kidneys, and digestive tract [1]. A skin biopsy is rarely needed [1]. Tests help rule out similar conditions, show how active the disease is, and find out whether organs other than the skin are involved [1]:
Blood tests: to measure autoimmune proteins and kidney function [1].
X-rays: to show changes in the skin, bones, and internal organs such as the lungs and intestines [1].
Swallowing tests: to check how well the esophagus works [1].
Pulmonary function tests: to make sure the lungs are working properly [1].
Echocardiography: an ultrasound of the heart to look at how it is functioning [1].
How is juvenile systemic scleroderma managed and treated?
The goal of treatment is to stop inflammation, keep the condition from getting worse, and prevent internal organ involvement [1].
Skin Protection and Lifestyle
Protecting the skin helps maximize blood flow to the skin, hands, and feet, especially for children with Raynaud's phenomenon. Helpful steps include:
Avoid injury to affected areas, especially the tips of the fingers and toes [1].
Protect hands and feet from cold: keep rooms warm and dress your child in an extra layer in winter, plus a hat with earmuffs, gloves, and warm socks. Wool is warmer than cotton or synthetic fabrics, and several thin layers work better than one thick layer [1].
Avoid smoking and exposing your child to smoke [1].
Avoid cold medicines that contain pseudoephedrine [1].
Protect your child from excess sun and use sunscreen as recommended [1].
Avoid astringents, body or facial scrubs, and harsh detergents on the skin [1].
Use lotions as prescribed by your child's healthcare provider to keep the skin soft [1].
Physical and Occupational Therapy
Basic stretching and guided exercise programs with physical and occupational therapists help a child keep flexibility, joint range of motion, muscle strength, and blood flow to affected areas [1]. Therapy also helps prevent joint contractures, which are joints that become permanently bent [1]. Splints may be recommended if needed [1].
Medicines
Your child's healthcare provider may recommend different medicines, including:
Corticosteroids: to decrease inflammation in the muscles, joints, and skin and to help treat early internal organ inflammation. They generally don't work in the later, fibrotic stages [1].
NSAIDs (such as ibuprofen and naproxen): sometimes used for arthritis to reduce joint inflammation, with care to avoid overuse when kidney function is reduced [1].
Other immune-modulating medicines: to lessen the autoimmune response and help prevent the inflammation and scarring that follow [1].
Medicines that widen blood vessels: to improve blood flow and treat Raynaud's phenomenon [1].
Other Options
In rare cases, orthopedic hand or cosmetic surgery may be needed to correct severe joint or skin deformities or scars, and the condition must first be in remission (not active) for several years [1]. A provider may also consider autologous bone marrow transplantation as an option, and other treatments are being studied [1].
What can families expect with juvenile systemic scleroderma?
Juvenile systemic scleroderma has no cure, but families can help a child manage the condition. It is a chronic, slowly developing disease that lasts for months or years [1], and the outlook depends on how widespread the symptoms are and how much of the body is involved [1]. Children with lung, heart, or kidney problems have the greatest risk of complications, and the condition can affect bone growth [1]. Although it often doesn't go away, it can stay at the same level without getting worse for several years [1]. Children should live as normally as possible, attend school, and take part in activities [1], and regular checkups are needed to watch for high blood pressure and lung, kidney, digestive, and heart problems [1].
What is the bottom line on juvenile systemic scleroderma?
A diagnosis of juvenile systemic scleroderma can feel overwhelming for any family. By working closely with a specialized medical team, following a dedicated care plan, and protecting the skin from cold and injury, parents can help their child protect their health and keep as normal a childhood as possible. If your child has fingers that change color in the cold, swollen or tight skin, or unexplained joint stiffness, talk with a healthcare provider.
Frequently Asked Questions
Is systemic scleroderma the same as localized scleroderma?
No. Localized scleroderma affects the skin in only some areas of the body, may spread to the muscles or bones underneath [1], and rarely involves internal organs [1]. Systemic scleroderma can thicken the skin in all parts of the body and cause scar tissue in internal organs such as the kidneys, heart, lungs, and digestive tract [1].
What causes juvenile systemic scleroderma?
The exact cause is unknown. Research suggests that injury to the cells lining the blood vessels over-activates the skin's connective tissue cells, which then make too much collagen [1]. Because the cause is unknown, there is no known way to prevent it [1].
Can a child grow out of scleroderma?
Systemic scleroderma is a chronic condition with no cure, and it often doesn't go away. It can, however, stay at the same level without getting worse for several years [1], and treatment aims to control inflammation and protect organs.
Why do my child's fingers change color in the cold?
This may be Raynaud's phenomenon, in which the fingers or toes turn white, blue, and then red with cold or stress [1]. It often appears early and can be a sign of systemic sclerosis [1], so ask a healthcare provider to take a look.
Is scleroderma contagious?
No. It is an autoimmune disease, which means the child's own immune system is involved. It cannot be passed to other people.
At what age does juvenile systemic scleroderma usually start?
It is rare in early childhood. Among all people with scleroderma, about 2% develop it before age 10 and about 7% between ages 10 and 19 [1]. In a North American registry, the median age at onset was 10.3 years [2].
Can scleroderma affect the heart?
Yes. Scar tissue can develop in internal organs, including the heart [1], which is why regular monitoring by specialists is needed. Heart involvement was more common in the limited subtype in one international cohort [3].
Can scleroderma cause high blood pressure?
Yes. When the kidneys are affected, kidney problems can lead to hypertension (high blood pressure) [1], so blood pressure is checked regularly [1].
What kind of doctor treats juvenile scleroderma?
A healthcare provider often teams up with a rheumatologist, a dermatologist, and specialists for the lungs, heart, kidneys, and digestive tract [1].
Can children with scleroderma play sports?
Children should take part in school and activities and live as normally as possible. In general, there are no limits on physical activity as long as it is safe [1], and exercise helps prevent deconditioning and builds strength, flexibility, and endurance [1]. Ask your child's care team what is right for your child.
How should I protect my child from the sun?
Protect your child from excess sun exposure and use sunscreen as recommended by your healthcare provider [1]. For Raynaud's phenomenon, cold is usually the bigger trigger, so keep hands and feet warm.
What are telangiectasias?
They are enlarged red blood vessels that show at the skin, most often on the hands, the face, and around the nail beds [1].
What is calcinosis?
Calcinosis refers to hard calcium deposits that can form under the skin or in other areas [1].
Can a child have scleroderma without knowing it?
Yes. Pain usually isn't severe, so it is common for children to have scleroderma without realizing it [1]. Swollen or tight skin and color changes in the fingers are signs worth noticing.
How often should my child see a healthcare provider?
Children with systemic sclerosis need regular checkups, especially to watch for high blood pressure and for lung, kidney, digestive, and heart problems [1]. See a provider right away for severe pain, trouble moving parts of the body, or symptoms that affect daily life [1].
What is the long-term outlook for children?
The outlook depends on how widespread the symptoms are and how much of the body is involved [1]. Children with lung, heart, or kidney problems have the greatest risk of complications [1], and life expectancy varies with severity, especially when organs are affected [1].
References
Cleveland Clinic. Juvenile Systemic Scleroderma. Cleveland Clinic. Updated August 5, 2022. https://my.clevelandclinic.org/health/diseases/4910-pediatric-systemic-scleroderma
Stevens BE, et al. Clinical Characteristics and Factors Associated With Disability and Impaired Quality of Life in Children With Juvenile Systemic Sclerosis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry. Arthritis Care Res (Hoboken). 2018;70(12):1806-1813. PMID 29457372. https://doi.org/10.1002/acr.23547
Foeldvari I, et al. Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort. Arthritis Care Res (Hoboken). 2022;74(10):1575-1584. PMID 33787070. https://doi.org/10.1002/acr.24609
Kaneko U, et al. Clinical and Epidemiological Features of Juvenile-Onset Systemic Sclerosis From a Nationwide Survey in Japan. J Rheumatol. 2026;53(4):425-434. PMID 40953948. https://doi.org/10.3899/jrheum.2025-0175
Medical disclaimer
This article is for general informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional for diagnosis and treatment decisions. See a provider promptly for severe pain, trouble moving, new breathing problems, or other symptoms that affect your child's day-to-day life.
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