Published on 18/03/2015 by admin

Filed under Dermatology

Last modified 18/03/2015

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Reed M. Garza and Heidi T. Jacobe

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports


Morphea, also known as ‘localized scleroderma’ (a term that should be discouraged because of unnecessary confusion with scleroderma among providers and patients), is an autoimmune disorder characterized by inflammation and subsequent sclerosis of the dermis and sometimes subcutis and beyond. Although previously considered self-limited, emerging evidence suggests a remitting relapsing course may be common. Further, untreated lesions may leave behind cosmetic and functional disfigurement, warranting treatment to avoid these sequelae. Morphea has a spectrum of manifestations ranging from skin only to internal involvement (musculoskeletal is most common) that is different from scleroderma. It can be classified into subtypes including circumscribed, linear or generalized. Any subtype may have involvement of the subcutis. Onset is bimodal and can occur in childhood where linear, morphea is most common or in adults where circumscribed and generalized predominate.

Management strategy

The management of morphea is challenging in large part due to the lack of standardized methods of evaluation and minimal evidence for the efficacy of many treatments. Studies conducted by rheumatologists and dermatologists underscore large discrepancies in the manner in which patients with morphea are evaluated and treated, and frequent use of therapy that is not supported by current evidence. Recently published works are addressing these gaps by providing consensus treatment plans, clinical outcome measures for use in practice, and evidence for therapeutic efficacy.

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.