Update on Management of Morphea (Localized Scleroderma) in Children

Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. The assessment of activity and damage can be done with a validated instrument like LoSCAT. Disease classified as “low severity” which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. Methotrexate is recommended for linear, deep and generalized morphea. Steroids are effective in the early inflammatory stage and used in combination with methotrexate. Methotrexate is continued for at least 12 months after adequate response is achieved. Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate. There are also reports of improvement of disease with ciclosporine and hydroxychloroquine. In severe cases, recalcitrant to standard therapy there may be a role for biologics, JAK inhibitors, and IVIG. Supportive measures like physiotherapy and psychiatric counseling are also important in the management of morphea. Orthopedic surgery and other measures like autologous fat transfer may be advocated once the disease is inactive.