Localized Scleroderma (Juvenile)

Localized scleroderma, called "linear" or "morphea", is an autoimmune condition that causes inflammation and hardening of skin and muscles in one part of the body. The inflammation triggers connective tissue cells to produce too much collagen, a fibrous protein found in many tissues. Excess collagen can lead to fibrosis, which is like scarring. Localized scleroderma can happen at any age. It affects about 50 out of every 100,000 children. It is more common in Caucasians. Most patients are female. Certain genes may increase a child’s risk of developing localized scleroderma. This condition often develops early in life between ages 6 and 10 years old.

What Are the Signs/Symptoms?

Skin lesions include linear forms (lines or streaks) and circumscribed morphea (round). Most children have lesions in one part of their body. Localized scleroderma may affect muscle and bone, and cause growth and joint problems in affected areas. Early signs include lesions that are red or purplish around the border. Some children have white, waxy, hard lesions. Skin hardening may cause discomfort, disfigurement, sores and limited joint movement. Linear lesions on the face could cause eye inflammation, eyelid or dental problems, headaches, seizures, or brain problems. A pediatric rheumatologist or dermatologist may diagnose the disease with a physical exam. A skin biopsy will confirm localized scleroderma.

What Are Common Treatments?

The goal of localized scleroderma treatment is to control inflammation and stop disease progression. Methotrexate (Rheumatrex, Trexall, Otrexup, Rasuvo), oral corticosteroids (Prednisone) or infused corticosteroids (Methylprednisolone given through a vein) suppress the immune system to control inflammation. Other oral medications used to treat localized scleroderma are mycophenolate mofetil (CellCept), cyclosporine (Neoral, Sandimmune) and tacrolimus (Prograf, Protopic, Hecoria). For refractory or severe cases, biologic therapies may be considered. These include TNF inhibitors such as infliximab (Remicade, Inflectra, Avsola, Ixifi, Renflexis), adalimumab (Humira), and etanercept (Enbrel). Other biologics used in treatment include abatacept (Orencia) and rituximab (Rituxan, Ruxience, Riabni). Additionally, immunomodulators such as Janus kinase (JAK) inhibitors and intravenous immunoglobulin (IVIG) have shown promise in case studies for select patients.

Mild skin lesions may be treated with phototherapy and topical agents such as corticosteroid creams, calcipotriene, tacrolimus, pimecrolimus, and imiquimod.

Living with Localized Scleroderma

Children with localized scleroderma should live as normally as possible. They should continue to go to school. Children with impaired ability to walk or write may need accommodation. Children should stay active, although those at risk for skin breakdown or with severe joint problems may need to limit contact sports. Physical and occupational therapy is especially important for children with limb length differences, limited joint movement or muscle weakness. Therapy also improves strength and prevents loss of joint flexibility and function. Cosmetics help make skin lesions on the face or limbs less noticeable. Regular eye and skin exams can spot serious complications that require treatment. Localized scleroderma can persist for years or recur after years of inactive disease.

Updated March 2025 by Kerry Hurlbut, DNP, CRNP, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified healthcare provider for professional medical advice, diagnosis and treatment of a medical or health condition.