Scleroderma is a group of rare diseases that involves the tightening and hardening of the skin and connective tissues of the body.
There are two main types of scleroderma:
- Localized scleroderma. This type of scleroderma includes morphea or linear; it primarily affects the skin.
- Systemic scleroderma. This type of scleroderma can affect many systems throughout the body.
The main symptoms of localized scleroderma include:
- Swelling of the skin or fingers
- Change in the color of the skin, especially when there is a change in air temperature
- Thickening or scar-like changes in the skin
- Tightness in the joints that can prevent normal movement of the arms, legs, hands and feet
- Loss of hair and sweat glands in the affected areas over time
Along with the skin-related symptoms above, symptoms of systemic scleroderma also include:
- Difficulty swallowing or food getting caught in the throat
- Gastroesophageal reflux
- Shortness of breath
- Dry cough
Rarely, systemic scleroderma can also affect proper function of the lungs, heart or kidneys, which can be life-threatening.
Diagnosis of Childhood Sclerodermas & Morphea
To diagnose scleroderma, your child's rheumatologist will conduct a comprehensive physical exam to check for the associated symptoms. The doctor may also recommend one or more of the following tests to help make a clear diagnosis:
- Blood test. Your child’s doctor may conduct a blood test to check for elevated levels of specific antibodies produced by the immune system.
- Biopsy. Your child’s doctor may examine a small tissue sample of the affected area under a microscope.
- Pulmonary function test. Your child’s doctor may perform breathing tests to check the function of your child's lungs, which can be affected by the condition.
- Computed tomography (CT) scan. Your child’s doctor may run this type of scan to analyze areas of the body that may be affected by scleroderma.
- Echocardiogram (EKG). Your child’s doctor may order an EKG, which checks the function of your child’s heart.
If your child is diagnosed with scleroderma, it is important to closely follow the treatment plan of the pediatric rheumatologist.
Treatments
Treatments
If your child has localized scleroderma, it is possible that the skin problems that are part of the condition will naturally fade away within three to five years. If your child has systemic scleroderma, the condition will likely become worse, and it is important to follow the prescribed treatment plan.
Pediatric rheumatologists at Riley at IU Health may prescribe one or more of the following medicines to treat localized scleroderma or systemic scleroderma:
- Immunosuppressants. These medicines reduce an overactive immune system and can help lessen the symptoms of scleroderma.
- Light therapy. This process treats the skin with ultraviolet light to help improve appearance.
- Corticosteroids. These medicines can help reduce inflammation and keep the skin from thickening.
- Calcipotriene cream. A synthetic form of vitamin D, this topical cream can help lessen the skin-related symptoms of scleroderma.
- Antimalarial drugs. Medicines such as hydroxychloroquine can help reduce inflammation as well as potentially slow the progress of the condition.
- Physical therapy. A physical therapist can work with your child to practice stretching, strengthening and range-of-motion exercises that can help him or her remain mobile and active.
If your child has systemic scleroderma, his or her doctor may prescribe acid-reducing medicines such as Prilosec to help with acid reflux symptoms. In the case of children who have suffered severe lung damage due to systemic scleroderma, a lung transplant may be necessary.
Key Points to Remember
Key Points to Remember
- Scleroderma is a group of rare diseases that involves the tightening and hardening of the skin and connective tissues of the body.
- The two main types of sclerodermas are localized scleroderma and systemic scleroderma.
- Scleroderma primarily affects the skin, though the systemic form of the disease can cause serious damage to the heart, lungs and kidneys.
- Your child’s doctor will likely prescribe a combination of medicines and physical therapy to help reduce the symptoms of scleroderma.
Support Services & Resources
Support Services & Resources
Visit the trusted websites below to learn more about childhood sclerodermas & morpheas.
Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.
The Scleroderma Foundation provides detailed information about juvenile scleroderma as well as patient resources and support.
The American College of Rheumatology provides education and support for children and families living with localized juvenile scleroderma
Locations
Locations
Locations
In addition to our primary hospital location at the Academic Health Center in Indianapolis, IN, we have convenient locations to better serve our communities throughout the state.
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