Morphea (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.

Approach to Therapy

• In general, active disease (present <3 months or with clinical signs of inflammation) is most responsive to treatment
• Sclerotic lesions are less likely to improve, and atrophic lesions rarely respond to therapy.
• topical therapies (eg, high potency topical corticosteroids, topical vitamin D, topical tacrolimus) are often used as initial treatments despite the paucity of studies to support their efficacy.
• There is more evidence for the efficacy of phototherapy, which also can be used as first-line therapy
• In patients with generalized morphea, topical therapy is often ineffective
• Phototherapy and systemic therapy are the preferred initial treatments

Systemic

• patients with inflammatory, rapidly progressive morphea (defined as rapid evolution to involve multiple body sites or rapid extension of lesions over multiple joints within less than six months) simultaneously with methotrexate and a systemic glucocorticoid (eg, intravenous methylprednisolone or oral prednisone)
• reserve monotherapy with methotrexate for those patients with less inflammatory and more slowly progressing disease and patients with contraindications to glucocorticoid therapy.
• Patients with superficial morphea who fail to improve with phototherapy may also be candidates for systemic treatment

Topical

• Topical therapies are unlikely to be effective for disease involving the subcutis or deeper tissues
• Not useful for preventing the development of new lesions in patients with rapidly progressive disease

Topical Tacrolinus 0.1%	·         may be effective for active, inflammatory morphea
Topical corticosteroids	·         high potency topical corticosteroids are widely used ·         usually applied twice daily or once daily with occlusion ·         If there is no response after 10 to 12 weeks, treatment should be discontinued.
intralesional corticosteroids	·         no formal studies of the efficacy ·         been effective for the treatment of circumscribed morphea or as adjuvant therapy for recalcitrant lesions ·         typically use triamcinolone acetonide at a concentration of 5 to 10 mg/mL. ·         In patients who respond to treatment, repeat injections every four to six weeks until sufficient improvement is attained
Topical vitamin D	·         Clinical improvement was noted in all patients during the study.
Imiquimod	·         Limited data suggest that imiquimod is effective in morphea

References:

1. www.uptodate.com
2. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma
3. http://www.dermnetnz.org/immune/systemic-sclerosis.html