Use of Extracorporeal Photopheresis in Scleroderma: A Review

Scleroderma is a heterogeneous group of diseases that can be localized or systemic. Localized scleroderma is a fibrosis of the skin characterized by inflammation and thickening due to excessive collagen deposition, and systemic sclerosis (SSc) is characterized by vasculopathy, immune dysregulation and skin fibrosis. In general, the prognosis of scleroderma highly depends on the degree of visceral involvement and relates to the degree of skin fibrosis. Despite the numerous therapies used for patients with scleroderma, the disease-related morbidity and mortality are high. Studies have explored the effects of extracorporeal photopheresis (ECP) in scleroderma treatment. Originally used in the treatment of cutaneous T-cell lymphoma, ECP is an immunomodulatory procedure in which a patient's white blood cells are treated with 8-methoxypsoralen and exposed to UVA radiation to inhibit cell proliferation and induce immunosuppression.