Circumscribed morphea | Generalized morphea | Linear scleroderma (trunk/limbs) | Linear scleroderma (head/face) | |
Clinical features | One or a few circumscribed sclerotic plaques with hypo- or hyperpigmentation and an inflamed violaceous border. | Individual plaques (four or more and larger than 3 cm) that become confluent and involve at least two anatomic sites. | Linear induration involving dermis, subcutaneous tissue, and sometimes muscle and underlying bone, and affecting the limbs and/or the trunk. | Linear induration affecting the face and/or scalp, involving underlying subcutaneous tissues, muscles, and bone. Underlying cerebral abnormalities have been reported. |
Treatment | Often unnecessary. Topical corticosteroids for active lesions. | Suppress inflammation with oral or IV glucocorticoids for three months in addition to methotrexate for at least 24 months. Mycophenolate mofetil can be used in methotrexate-refractory cases. | Suppress inflammation with oral or IV glucocorticoids for three months in addition to methotrexate for at least 24 months. Mycophenolate mofetil can be used in methotrexate-refractory cases. | Suppress inflammation with oral or IV glucocorticoids for three months in addition to methotrexate for at least 24 months. Mycophenolate mofetil can be used in methotrexate-refractory cases. |
Prognosis | Good prognosis lesions less active within three years, but pigmentary changes often persist. | Generally improves within five years of onset, although textural and pigmentary changes can remain. | Long-term effects minimized by effective suppression of the inflammatory process. Ultimately the disease tends to resolve, but it can remain active for many years. | Scarring, growth defects, and alopecia persist, but inflammatory component usually resolves. |
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