Scleroderma. It’s a word many people haven’t heard before, one that sounds more like a dinosaur name than a medical condition. In fact, the term is ancient in one sense: it comes from Greek words for “hard skin” (“skleros” meaning “hard,” and derma meaning “skin”). Patients with scleroderma have been described in ancient texts dating as far back as Hippocrates in 400 BC! Scleroderma is well-known for how it hardens the skin, but it is much more than that. The good news is that new treatments are improving the lives of patients with this “dinosaur disease.”
Each person’s skin is unique, so scleroderma looks different for every patient. A child or young adult may start out with a small area of affected skin that changes in texture. Some people describe the skin as becoming tighter, firmer, more stretched, or thickened. The affected area may lighten, darken, or develop a red border.
It can take a long time searching to find the right diagnosis. On average, it takes 12-15 months from the first symptoms to being correctly diagnosed with scleroderma. This is partly because scleroderma is relatively rare, affecting about 3 to 4 out of every 10,000 children, and partly the reason the condition can be difficult to identify, especially in the early phases. Scleroderma is an autoimmune disease, which means that it is caused by the body’s immune system attacking the skin, joints or internal organs.
There are several different subtypes of scleroderma, which can be grouped into localized and systemic forms.
Localized Scleroderma
“Localized” means that the condition only affects the skin. Children affected with scleroderma most often have this form. Localized scleroderma can pop up on any part of the skin, but common areas include the forehead, arms, legs, and trunk. It usually starts as an area where the skin becomes red, shiny, tight, hard or thickened and can spread down an arm, leg or face. Sometimes it starts on the scalp and causes hair loss. It can even look like a bruise. If it crosses a joint on the arm or leg, the child may have trouble straightening it. Children with this type of scleroderma are often referred to a dermatologist who may prescribe steroid creams. However, most children with localized scleroderma need medications that work throughout the body (like pills or injections) to treat the disease and prevent long term damage and growth disturbances.
Some words often used in the diagnosis for the localized form include:
Morphea
Linear scleroderma
Scleroderma “en coup de sabre” (from the French meaning “cut of a sword”)
Systemic Scleroderma
“Systemic” means that the condition causes problems in multiple organ systems of the body, such as the skin, lungs, heart, digestive system, or kidneys. Fortunately, this form is very uncommon in children. Over time, this type of scleroderma can lead to permanent damage in the lungs, heart or kidneys.
Some words associated with the systemic form include:
Limited scleroderma
CREST Syndrome
Diffuse scleroderma
If you or your child’s doctor suspect they may have scleroderma, it is important to see a pediatric rheumatologist right away. Scleroderma can spread over time, especially if it is not treated. If a large area is affected, a child’s body may not grow correctly. For example, scleroderma on the leg could cause one leg to be shorter than the other, or cause the knee to not bend fully. Scleroderma on the forehead or scalp could lead to hair loss and disfigurement.
While there is no cure for scleroderma, treatments are available. There are medications that can slow or stop the progression of the disease, and other therapies to address the specific symptoms that scleroderma causes.
If your child is diagnosed with scleroderma, your family should work closely with a pediatric rheumatologist to find the right treatments to help them thrive and treat their disease. Some treatments that are effective include steroids, “disease-modifying” medications, or newer biologic medications that are also used to treat arthritis. Your child will need to follow up with their pediatric rheumatologist regularly and will need blood tests several times per year to monitor their condition and check for side effects from the medications. Your child’s doctor will work with you as a team to limit side effects from medications and support them through school and activities such as sports. With timely treatment, children with scleroderma can grow to become adults who go to college, work and lead full lives.
Researchers are working hard to make pediatric scleroderma become extinct through collaborations between multiple centers, like the research network called Childhood Arthritis and Rheumatology Research Alliance (CARRA) and research grants from the National Scleroderma Foundation. In the meantime, many children and young adults are able to lead fulfilling lives while keeping their scleroderma under control.
Elizabeth Murray is a pediatrician at Nationwide Children's Hospital.
Vidya Sivaraman, MD
Vidya Sivaraman, MD, is a pediatric rheumatologist at Nationwide Children's Hospital and Associate Professor of Clinical Pediatrics at The Ohio State University. She is the Associate Fellowship Director of the Rheumatology Fellowship Program and Director of Quality Improvement in Rheumatology. Her research interests include pediatric ANCA-associated vasculitis, Localized Scleroderma, and vaccination in immunocompromised children.
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