Initial evaluation

The initial evaluation of patients with morphea should involve:

Active morphea lesions should be treated as the lesions are frequently symptomatic and can produce permanent cosmetic and functional sequelae. Initial evaluation should be focused on determining the extent, severity, and activity of morphea lesions laying the groundwork for rational therapeutic choices.

Treatment strategy

Once the evaluation is complete, treatment decisions should be based on activity and damage, depth of involvement (dermal versus deep structure), area of involvement, and disease progression.

In general, limited dermal inflammatory lesions that are not rapidly progressing nor cosmetically or functionally threatening may be treated with topical therapies (calcipotriene, calcitriol, tacrolimus, or topical steroids) or intralesional triamcinolone. These patients should be closely followed, and, if their lesions multiply or spread, suppressive therapy is indicated (phototherapy or systemic steroids and methotrexate) to prevent continued development of new lesions. In general, extensive active lesions involving the subcutis or below, or those that are cosmetically or functionally threatening such as en coup de sabre or hemifacial atrophy should receive methotrexate and systemic steroids. Widespread active dermal morphea may be treated with phototherapy, preferably UVA1, or if unavailable UVA without psoralen. Serial photography or skin scores are invaluable to determine efficacy.

Treatments not supported by current evidence

The evidence currently does not support the use of topical steroids, oral or topical calcipotriol, D-penicillamine, interferon-γ, or antimalarials. Therefore, the use of these treatments is discouraged in severe cases of morphea where function or cosmesis is threatened. Penicillamine also has a significant side effect profile including nephrotoxicity, and should be avoided.