Scleroderma in Children
Scleroderma is an ongoing (chronic) disease that causes abnormal growth of connective
tissue. It can affect the joints, skin, cartilage, and internal organs. It is a rare
degenerative disease and gets worse over time.
are 2 main types of scleroderma: localized and systemic:
Localized scleroderma. There are 2 forms:
Morphea. This type
causes 1 or more hard, oval-shaped, or irregular patches on any part of the
body. Patches are whitish or darkened and sometimes have a purple or
Linear. Lines or
streaks of thickened skin form over 1 or more areas of the body, such as an
arm, leg, or the head. Joint symptoms may also occur, such as pain,
stiffness and trouble moving joints.
Systemic scleroderma. This more severe type can affect
the whole body. This type is more common in adults and rarely occurs in
Scleroderma is thought to be an autoimmune disease. This means the symptoms are caused
by the body attacking its own healthy tissues. Genes play a role in the disease, but
it is not passed on from parents to children. Some environmental factors may also
play a role.
Scleroderma can lead to scarring of the skin, joints, and internal organs. Symptoms
occur a bit differently in each child. They may include:
- Thick, swollen fingertips
- Pale and tingly fingers that may turn numb when out in the cold or during emotional
upset (Raynaud's phenomenon)
- Joint pain
- Taut, shiny, darker skin on large areas, that may cause problems with movement
- Spider veins
- Calcium bumps on the fingers or other bony areas
(immobile) fingers, wrists, or elbows because of scarring, hardening and tightness
- Sores on fingertips and knuckles
- Grating noise as inflamed tissues move
- Scarring of the esophagus, leading to heartburn and trouble swallowing
- Scarring of the lungs, leading to shortness of breath
- Heart failure and abnormal heart rhythms
- Kidney disease
Symptoms of localized sclerosis may include:
- Shiny, thickened patches of skin
- Lighter or darker (discolored) skin
- Joint tightness
These symptoms can seem like other health conditions. Make sure your child sees their
healthcare provider for a diagnosis.
child’s healthcare provider will ask about your child’s health history and do a physical
exam. Diagnosis is based on the changes in the skin and internal organs. An antibody
test may help show the type of scleroderma. Your child may need other tests, such
These can help find any problems with blood counts, or kidney or
Electrocardiogram (ECG). This test records the heart’s electrical activity,
shows abnormal rhythms, and finds heart muscle damage. An ECG may be done to find
changes in the heart muscle tissue due to scleroderma.
Echocardiogram. This test uses sound waves to create a moving image of the
heart and its valves. It looks at the structure and function of the heart.
This test uses a small amount of radiation to create images of internal tissues,
bones, and organs. X-rays may show changes in bone, soft tissues, and organs caused
Treatment will depend on your child’s symptoms, age, and general
health. It will also depend on how severe the condition is.
Treatment may include:
to ease pain. These include nonsteroidal anti-inflammatory drugs or
- Medicine to slow the skin thickening process and delay damage to internal organs
to weaken the immune system (immunosuppressive medicines)
- Treatment of specific symptoms, such as heartburn and Raynaud's phenomenon
therapy and exercise, to maintain muscle strength
with your child’s provider about the risks, benefits, and possible side effects of
Complications of scleroderma vary depending on the type of the disease and how severe
it is. Treatment may prevent or reduce problems. Possible complications may include:
- Thickened, tight skin
- Behavior and learning problems
- Vision changes
(gastroesophageal reflux) or heartburn
- Less lung function
- Heart and kidney damage
Work with your child's healthcare provider. Together you can make a treatment plan.
The plan can help your child be active as much as possible in school, and in social
and physical activities. Make life as normal as possible for your child. Encourage
exercise and physical therapy and find ways to make it fun. You can also help your
child find a support group to be around other children with scleroderma. Work with
your child's school to make sure your child has help as needed. Your child may also
qualify for special help under Section 504 of the Rehabilitation Act of 1973.
When to Call a Healthcare Provider
Tell the healthcare provider if your child's symptoms get worse or there are new symptoms.
- Scleroderma causes abnormal growth of connective tissue. It can affect the joints,
skin, and internal organs.
- Scleroderma can affect 1 area of the body or affect the whole body. It is less
common in children for it to affect the whole body.
- Localized scleroderma may affect patches of the skin on the torso, arms, legs, or
is no known cure for scleroderma. Treatment is focused on relieving pain and slowing
down further damage to the body.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the
visit, write down the name of a new diagnosis, and any new medicines, treatments,
tests. Also write down any new directions your provider gives you for your
- Know why
a new medicine or treatment is prescribed and how it will help your child. Also know
what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
how you can contact your child’s healthcare provider after office hours. This is
important if your child becomes ill and you have questions or need advice.