Scleroderma is an extremely rare autoimmune disorder that affects approximately 250 out of every one million Americans. It is estimated that there are around 5,000 to 7,000 children currently diagnosed with it in the United States.
The word scleroderma literally translates to ‘hard skin’.
This condition is known for dense, thick scar tissue that develops on the skin, joints, and sometimes internal organs due to the body’s immune system attacking healthy cells and tissue. The body then adversely reacts by creating an excess amount of collagen which is secreted into the skin and organs causing the characteristic hardening.
Some common symptoms of scleroderma include:
- Tightness of the skin
- Decreased function in the hands, particularly around the fingers and wrist
- Joint or muscle pain, inflammation and stiffness
- Chronic fatigue
While there is currently no cure for scleroderma, there are many effective treatments that board-certified pediatric rheumatologist Dr. Ruy Carrasco and pediatric nurse practitioner Shelby Brooks, CPNP-PC use every day to help our young patients at Pediatric Rheumatology Consultants live more active, comfortable lives.
In honor of June being Scleroderma Awareness Month, here are 10 fast facts to know about the disease:
- There are two forms of pediatric scleroderma: localized and systemic.
- Localized scleroderma is more common in children, typically only affecting the skin.
- Despite its outward and often concerning appearance, scleroderma is not contagious and cannot be passed on to someone else.
- Approximately two-thirds of children with scleroderma are girls.
- There is no evidence that scleroderma is more prevalent in one race over another.
- The average age for developing pediatric scleroderma is between seven and eight years old.
- Localized scleroderma typically progresses quickly in its early stages but often stops spreading after three to four years.
- There’s no cure for scleroderma, but it’s possible to manage the symptoms so the disease doesn’t progress.
- Children with systemic sclerosis have a higher risk for internal organ damage and should be monitored very closely.
- Sometimes Raynaud’s Disease/Phenomenon also occurs in patients with scleroderma, causing the body to experience extreme sensitivity to cold and abnormal temperatures.
A definitive diagnosis for scleroderma is made by a pediatric rheumatologist in conjunction with a dermatologist via observations of the skin, blood tests, X-rays and additional scans to examine internal organs.
Our goals in managing scleroderma are to minimize inflammation, keep the skin and joints comfortable, and prevent any spreading of the disease to internal organs as much as possible.
In children, localized scleroderma can typically be treated with:
- Creams and ointments
- Steroid medications to reduce inflammation on the skin and in muscles and joints
- Physical therapy to promote flexibility
- Enhanced and dedicated skin protection from sun exposure, extreme temperatures, and harsh chemicals
If scarring is found on the internal organs from systemic scleroderma, surgery may be needed to remove the damaged tissue.
Our outlook for children living with scleroderma is generally positive provided that they manage their symptoms diligently and are seen often by their pediatric rheumatologist.
We fully encourage our scleroderma patients to stay active and participate in as many sports and extracurriculars that they choose to enjoy.
Pediatric Rheumatology Consultants treats a wide range of autoimmune disorders and diseases of the musculoskeletal system in children and teenagers. Our team includes the expertise of Dr. Carrasco and Shelby Brooks, CPNP-PC along with the compassionate care of our experienced nurses and infusion nurses. To schedule an appointment with us, please click here.
