Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes in the skin. Patients present with single or multiple inflammatory or sclerotic plaques. Disease activity typically persists for three to six years, although some patients develop more persistent or recurring episodes of activity.
The majority of adults with morphea are managed with observation, topical medications, or phototherapy. The level of disease activity, depth of involvement, body surface area involved, and the potential for functional impairment or cosmetic disfigurement determine the most appropriate approach to treatment. Aggressive systemic therapy is warranted for patients with active deep morphea or with lesions that are likely to cause significant cosmetic disfigurement, joint contractures, or other functional impairment. Occupational and physical therapy are additional important interventions for patients who have or are at risk for physical limitations.
Pharmacologic therapy, phototherapy, and other interventions for morphea in adults will be reviewed here. The pathogenesis, clinical manifestations, and diagnosis of morphea and the features and management of morphea in children are discussed elsewhere. (See "Pathogenesis, clinical manifestations, and diagnosis of morphea (localized scleroderma) in adults" and "Localized scleroderma in childhood".)
APPROACH TO THERAPY
A variety of treatment options are available for patients with active lesions of morphea; however, evidence in support of many of these therapies is limited. An algorithm for the treatment of patients with morphea is provided (algorithm 1).
The expected outcome of successful therapy for morphea is not the return to completely normal skin, although this may occur in very early inflammatory lesions. Therapeutic success for individual lesions is defined by improvement or resolution of erythema, lesion softening, or local hair regrowth. In patients with progressive disease, successful treatment arrests the formation of new lesions and limits disease spread. We have found that serial photographs are valuable for following the response to therapy.