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    Phototherapy in systemic sclerosis: Review.
    Photodermatol Photoimmunol Photomed 2017 Jul 13. Epub 2017 Jul 13.
    Division of Dermatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
    Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Skin sclerosis can cause disability, leading to decreasing quality of life. Various systemic antifibrotic therapies have been used; however, most have unsatisfactory results. Recently, phototherapy and in particular ultraviolet A (UVA) has been used to treat skin sclerosis in SSc patients with satisfactory results. The main mechanisms include lymphocyte apoptosis, cytokine alteration, inhibition of collagen synthesis and increased collagenase production, and neovascularization, leading to the breakdown of collagen fibrils resulting in skin softening or even healing digital ulcers. Most studies reported that psoralen plus UVA (PUVA) and UVA1 phototherapy improved clinical outcomes vis-à-vis skin sclerosis, joint mobility, ulcers, and histopathology. PUVA and UVA1 phototherapy therefore have potential as an alternative or adjunctive therapy for patients with SSc.

    Similar Publications

    Phototherapy: a promising treatment option for skin sclerosis in scleroderma?
    Rheumatology (Oxford) 2006 Oct;45 Suppl 3:iii52-iii54
    Department of Dermatology, University of Münster, D-48149 Münster, Germany.
    In systemic sclerosis (SSc; scleroderma) fibrosis of the skin can lead to considerable morbidity. No significant improvement has been reported from studies investigating antifibrotic therapies so far. In dermatology, phototherapy with ultraviolet (UV) irradiation is successfully used for treatment of several diseases because of its anti-inflammatory as well as immunosuppressive mechanisms, and its low-risk profile. Read More
    Ultraviolet A1 (340-400 nm) phototherapy for scleroderma in systemic sclerosis.
    J Am Acad Dermatol 2000 Oct;43(4):670-4
    Department of Dermatology, Nagoya City University Medical School, Nagoya, Japan.
    Background: The presence of an inflammatory infiltrate consisting of helper T cells and a dysregulated matrix metabolism leading to excessive deposition of collagen are two pathogenetic factors responsible for the developments of fibrosis and sclerosis in patients with systemic sclerosis. In previous studies, ultraviolet A1 (UVA1) radiation phototherapy was shown to deplete skin-infiltrating T cells through the induction of T-cell apoptosis and to up-regulate the expression of matrixmetalloproteinase-1 (collagenase-1) in dermal fibroblasts.

    Objective: Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis. Read More
    Ultrastructural changes induced in cutaneous collagen by ultraviolet-A1 and psoralen plus ultraviolet A therapy in systemic sclerosis.
    J Dermatol 2008 Feb;35(2):63-9
    Department of Geriatric and Environmental Dermatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
    In the present study, we examined the ultrastructural alterations in collagen fibrils clinically softened by ultraviolet-A1 (UVA1, 340-400 nm) therapy and psoralen plus long-wave ultraviolet (PUVA) therapy and compared collagen fibril diameters in four patients with systemic sclerosis (SSc). In skin sclerosis, the dermis is compacted from the epidermal layer to the sweat glands, and the collagen bundles are thicker with decreased space between them. We obtained skin specimens before and after UVA1 or PUVA therapy, and compared cutaneous alterations in one diffuse-type patient and one limited-type patient following UVA1 therapy, and in two diffuse-type patients following PUVA treatment. Read More
    UVA1 a promising approach for scleroderma.
    Am J Transl Res 2017 15;9(9):4280-4287. Epub 2017 Sep 15.
    Institute of Photomedicine, Shanghai Skin Disease Hospital, Tongji University School of MedicineShanghai, China.
    Scleroderma is a complex connective tissue disease characterized by fibrosis, vasculopathy, and immune system dysfunction. The heterogeneity of disease presentation and poorly understood etiology has made the management of scleroderma difficult. The available treatment options like immunosuppressive agents are associated with potentially hazardous side effects and physiotherapy, which to a certain degree helps to minimize the loss of function in digits and limbs, has only limited success. Read More