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Juvenile Scleroderma

The name scleroderma is a combination of the Greek words for "hard" and "skin." For many children, this is the main manifestation of this disease. Some children have a form of scleroderma where the skin all over their body changes and they may have internal organ involvement (juvenile systemic sclerosis). Others have a form where only a small part of the skin is affected (known as localized scleroderma, or LS). The term "morphea" may also be used to describe localized skin disease.

The cause of scleroderma and the relationship between the two types is not fully understood, though people with one type do not typically go on to develop the other. Genetics, the environment, and the cells of the blood, skin, and immune system all likely play a role. Both localized scleroderma and systemic sclerosis are rare diseases, though localized scleroderma is more common in childhood.

Symptoms

Children with localized scleroderma can have a range of skin involvement, from small spots that can look like birthmarks, to bigger lesions covering a large part of the limbs, trunk or face. Skin affected by scleroderma is typically waxy or hardened in texture, and may look reddish or purple. There may also be changes in the fat and bone underneath the skin lesion. Children who have localized scleroderma on their legs or arms may have one limb that looks smaller than the other, or may have trouble moving that limb if the hardened skin crosses a joint. Localized scleroderma may also affect the face, and in the most severe form of facial involvement, known as Parry-Romberg syndrome, the brain, eyes, teeth and tongue may be affected as well.

In children with juvenile systemic sclerosis (JSSc), the skin on the hands and face is usually affected in a symmetric pattern. The onset of JSSc is typically very gradual, and can therefore be hard to recognize. For many children, it can take years before the correct diagnosis is made. In addition to tightening and hardening of the skin, children may also develop calcinosis, or white deposits of calcium, underneath the skin. Raynaud’s phenomenon is common in JSSc, and is often the first symptom. This is characterized by episodes of white, blue, and red color changes to the fingers, toes, or even tip of the nose. Involvement of other organs may cause trouble swallowing, shortness of breath, joint pain, or heart, lung and kidney issues.

Diagnosis

Because both forms of scleroderma are rare diseases, they may be misdiagnosed for months, or even years. There is no single blood test that can tell if a child has scleroderma, though in JSSc, certain antibodies such as ANA and Scl-70 are often positive. Bloodwork is typically normal in localized scleroderma, but often, a pediatric rheumatologist can make the diagnosis based on history and physical exam alone. If the diagnosis is unclear, your child’s doctor may recommend a small skin biopsy, which is typically performed by a dermatologist. Depending on where the lesion is and whether or not there is concern for JSSc, your child’s doctor may suggest other workup as well, including an MRI, lung and heart function testing, or visits with other subspecialists.  

Treatment

Treatment for localized scleroderma depends on the extent of the skin involvement. Children with small, mild lesions may require only topical skin creams, but many children will need treatment with immunosuppressive medications such as methotrexate and steroids. In some patients, localized scleroderma will become inactive over a few months or years, and at that point, the medication may be safely weaned with close monitoring.

Children with JSSc often require long term treatment with immunosuppressive medications. Treatment for JSSc is guided by disease activity and severity, as well as which organs are involved. For both types of scleroderma, a multidisciplinary approach, with a focus on skin care, physical therapy, and patient education, is crucial to maintaining range of motion and flexibility.

For additional information and resouces visit Scleroderma Foundation.

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