PATIENT FACT SHEET Localized Scleroderma (Juvenile)

Localized scleroderma (“hard skin”) is an of many tissues. Excess collagen can lead to fibrosis, autoimmune condition that causes hardening of the which is like scarring. skin and muscles in one part of the body. There are Localized scleroderma is more common in Caucasians, several types of scleroderma; the localized form is also and can happen at any age. Most patients are female. called “morphea.” Certain genes may increase a child’s risk of developing Localized scleroderma causes inflammation in the skin, localized scleroderma. It affects about 50 out of every CONDITION which triggers connective tissue cells to produce too 100,000 children. DESCRIPTION much collagen, a fibrous protein that is a major part

In localized scleroderma, excess collagen leads to Localized scleroderma could cause disfigurement. Skin fibrosis, or tissue scarring. Skin lesions include linear hardening may cause discomfort, sores and limited forms (lines or streaks) and circumscribed morphea joint movement. Linear lesions on the face could (round). Most children have disease in one area or part of cause eye inflammation, eyelid or dental problems, their body. Localized scleroderma may affect muscle and headaches, seizures or brain problems. bone, and cause growth and joint problems in affected A pediatric rheumatologist or dermatologist may areas. Early signs include lesions that are red or purplish diagnose the disease with a physical exam. Tests can around the border. Some children have white, waxy, evaluate inflammation and rule out other causes. A SIGNS/ hard lesions. SYMPTOMS skin biopsy will confirm localized scleroderma. Imaging scans can measure bone and tissue abnormalities.

The first goal of treating localized scleroderma is cyclosporine (Neoral, Sandimmune) and tacrolimus to control inflammation with systemic medications. (Prograf, Protopic, Hecoria). Inflammation is the cause of the skin lesions and joint Mild skin lesions may be treated with topical agents, problems. Injected or oral methotrexate (Rheumatrex, such as corticosteroid creams, as well as phototherapy. Trexall, Otrexup, Rasuvo), oral corticosteroids Early on, children should have physical and occupational (prednisone) or infused corticosteroids (intravenous therapy to improve strength and function, and prevent methylprednisolone) may suppress the immune loss of joint flexibility and function. Therapy is especially COMMON system to control inflammation. important for children with limb length differences, TREATMENTS Other medications that may be used to treat localized limited joint movement or muscle weakness. Surgery is scleroderma are mycophenolate mofetil (CellCept), only needed for children with severe lesions, pain or limitation.

Children with localized scleroderma should live as to limit contact sports. Cosmetics help make skin lesions normally as possible. They should continue to go on the face or limbs less noticeable. to school. Children with severe disease, who may Patients need yearly check-ups with their pediatric have impaired ability to walk or write, may need rheumatologist to ensure that treatments are accommodations or therapy to adapt movements. controlling their inflammation. Localized scleroderma CARE/ Children should stay active, although those at risk for can persist for years or recur after years of inactive MANAGEMENT skin breakdown or with severe joint problems may need disease. Regular eye and skin exams can spot serious TIPS complications early so treatment can be prescribed.

Updated March 2017 by Sangeeta Sule, MD and Jennifer Cooper, MD and reviewed by the American © 2017 American College of Rheumatology College of Rheumatology Committee on Communications and Marketing. This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.