LINEAR MORPHEA

By Gary M. White, MD

Linear scleroderma


Linear scleroderma (linear morphea) is a form of localized scleroderma characterized by sclerotic lesions distributed in a linear, band-like pattern. It is more common in children but occurs in adults as well.

Clinical

A band-like linear induration, often with hypo or hyperpigmented areas, most commonly on the leg but also arm and forehead (en coup de sabre) is characteristic. A deep component with fixation to underlying structures may be present. Joint pain is quite common and joint contractures due to skin and tissue involvement may occur. Twenty percent of patients have onset by age 10; 75% by age 40. An underlying sclerosing bone dysplasia called melorheostosis can occur with the X-ray appearance said to resemble dripping candle wax. ANA may be strongly positive but often the diagnosis is made clinically.

Treatment

See morphea in a child. In the situation of joint contraction, physical therapy may be helpful. Assessing and monitoring for limb length discrepancies is important in those cases involving an extremity.

In one study of adults with linear morphea [JAAD 2016;74;577], delay in diagnosis and permanent functional limitations were common. Referrals for physical therapy, neurologic imaging, and evaluation of ocular involvement were underused. Treatment regimens incorporating methotrexate were more likely to result in resolution and less likely to result in progression or reactivation.

Additional Pictures

Linear scleroderma

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