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Therapeutic Approach

Morphea, also known as localized scleroderma, is an inflammatory, sclerosing disorder of the skin and soft tissue. Morphea is distinct from a related disorder: systemic sclerosis (scleroderma), which is an autoimmune connective tissue disorder with distinct systemic manifestations. Morphea can be divided into several subtypes, including linear, generalized, plaque (circumscribed), mixed, and pansclerotic. All subtypes of morphea transition through an early inflammatory stage followed by sclerosis and subsequent atrophy. Current therapeutic approaches are primarily aimed at ameliorating the inflammatory, or active stage of the disease and have minimal effect on inactive disease. Occupational and physical therapy are important interventions in the inactive stage, especially if there is sclerosis traversing joints putting patients at risk for disability. Surgical interventions can be also used to abrogate cosmetic and functional sequelae of disease damage, but the authors recommend these be performed after disease has been first treated medically with proven disease inactivity in collaboration with the appropriate surgical specialist (plastic surgeon or similar). The optimal management approach should be based upon disease activity, extent, depth, and risk of complications rather than on clinical subtype. In the authors’ experience, many patients can be managed with observation, topical medications (high-potency topical steroids such as clobetasol propionate preferred), or phototherapy (UVA1 preferred, but NBUVB can also be used) when morphea lesions are limited in distribution and spare the subcutis and soft tissue. Systemic therapy is warranted for patients with moderate to severe, active morphea that is rapidly progressing or causing significant cosmetic/functional impairment. First-line immunosuppressive therapy is methotrexate in conjunction with short courses of systemic steroids given over 3 to 4 months. Other agents, such as mycophenolate mofetil, are usually reserved for methotrexate-resistant cases or in those who cannot tolerate methotrexate.

In the pediatric population, principles of treatment are similar. However, children and adults affected by linear morphea, particularly of the head and neck, warrant special consideration. In this group of patients, the eyes, oral cavity, and central nervous system may be affected. This warrants careful history, physical examination, and review of systems and referral to an appropriate specialist familiar with morphea for treatment. Similarly, the musculoskeletal system may be affected either underlying the cutaneous linear morphea lesion or outside the affected area. In these cases, a collaborative approach with rheumatology is advised. See Table 49-1.

Table 49-1Morphea Treatment Table

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