BACKGROUND: Morphea (localized scleroderma) is a skin disorder with significant morbidity. No consistent recommendations exist for therapy, impeding patient care. OBJECTIVE: We sought to create an evidence-based therapeutic algorithm. METHODS: We reviewed English-language literature using search engines and hand searches for therapeutic interventions in morphea. Results were summarized. RESULTS: Narrowband ultraviolet B is appropriate for progressive or widespread superficial dermal lesions; broadband ultraviolet A/ultraviolet A-1 is appropriate for widespread or progressive deeper dermal lesions. Systemic treatment with methotrexate, corticosteroids, or both is indicated for deep or function-impairing lesions and rapidly progressive or widespread (severe) disease. Topical treatment with calcipotriene or tacrolimus is supported for limited, superficial, inflammatory lesions. Use of oral calcipotriol, D-penicillamine, interferon gamma, and antimalarials is not supported. LIMITATIONS: Limitations are publication bias; lack of adequately powered, controlled trials; and no validated outcome measures. CONCLUSION: Phototherapy, methotrexate/systemic corticosteroids, calcipotriene, and topical tacrolimus have the most evidence for efficacy in morphea. Treatment works best in inflammatory disease. Disease activity, severity, progression, and depth should play a role in therapeutic decision making.
BACKGROUND: Morphea (localized scleroderma) is a skin disorder with significant morbidity. No consistent recommendations exist for therapy, impeding patient care. OBJECTIVE: We sought to create an evidence-based therapeutic algorithm. METHODS: We reviewed English-language literature using search engines and hand searches for therapeutic interventions in morphea. Results were summarized. RESULTS: Narrowband ultraviolet B is appropriate for progressive or widespread superficial dermal lesions; broadband ultraviolet A/ultraviolet A-1 is appropriate for widespread or progressive deeper dermal lesions. Systemic treatment with methotrexate, corticosteroids, or both is indicated for deep or function-impairing lesions and rapidly progressive or widespread (severe) disease. Topical treatment with calcipotriene or tacrolimus is supported for limited, superficial, inflammatory lesions. Use of oral calcipotriol, D-penicillamine, interferon gamma, and antimalarials is not supported. LIMITATIONS: Limitations are publication bias; lack of adequately powered, controlled trials; and no validated outcome measures. CONCLUSION: Phototherapy, methotrexate/systemic corticosteroids, calcipotriene, and topical tacrolimus have the most evidence for efficacy in morphea. Treatment works best in inflammatory disease. Disease activity, severity, progression, and depth should play a role in therapeutic decision making.
Authors: Tamás Constantin; Ivan Foeldvari; Clare E Pain; Annamária Pálinkás; Peter Höger; Monika Moll; Dana Nemkova; Lisa Weibel; Melinda Laczkovszki; Philip Clements; Kathryn S Torok Journal: Eur J Pediatr Date: 2018-05-04 Impact factor: 3.183
Authors: Anagha Bangalore Kumar; Elizabeth K Blixt; Lisa A Drage; Rokea A El-Azhary; David A Wetter Journal: J Am Acad Dermatol Date: 2019-01-29 Impact factor: 11.527