Abstract
Localized scleroderma is an inflammatory disorder affecting the skin and underlying tissues, a certain subset of which develops other autoimmune diseases on the basis of a prominent autoimmune background. We here report a unique case of linear scleroderma presenting with a sclerotic plaque on the left thigh, multiple lymphadenopathy in bilateral inguinal and para-aortic lymph nodes, and hepatosplenomegaly, who later developed polymyositis. We describe the detailed disease course of our case and discuss the clinical significance of multiple lymphadenopathy in localized scleroderma based on a review of published work.
Keywords:
hepatosplenomegaly; immune abnormality; linear scleroderma; lymphadenopathy; polymyositis.
© 2016 Japanese Dermatological Association.
MeSH terms
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Autoantibodies / blood
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Autoimmune Diseases / diagnostic imaging
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Autoimmune Diseases / drug therapy
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Autoimmune Diseases / immunology
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Autoimmune Diseases / pathology*
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Centrioles / immunology
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Electromyography
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Fascia / diagnostic imaging
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Fascia / pathology
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Female
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Glucocorticoids / therapeutic use
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Hepatomegaly / diagnostic imaging
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Hepatomegaly / immunology
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Humans
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Lymph Nodes / pathology
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Lymphadenopathy / diagnostic imaging
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Lymphadenopathy / drug therapy
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Lymphadenopathy / immunology
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Lymphadenopathy / pathology*
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Lymphedema / diagnostic imaging
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Lymphedema / immunology*
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Magnetic Resonance Imaging
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Middle Aged
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Muscle, Skeletal / diagnostic imaging
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Muscle, Skeletal / enzymology
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Muscle, Skeletal / pathology
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Polymyositis / diagnostic imaging
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Polymyositis / drug therapy
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Polymyositis / immunology
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Polymyositis / pathology*
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Prednisolone / therapeutic use
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Scleroderma, Localized / diagnostic imaging
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Scleroderma, Localized / drug therapy
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Scleroderma, Localized / immunology
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Scleroderma, Localized / pathology*
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Skin / pathology
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Splenomegaly / diagnostic imaging
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Splenomegaly / immunology
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Tomography, X-Ray Computed
Substances
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Autoantibodies
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Glucocorticoids
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Prednisolone