Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characte...Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characterized by myositis,arthritis,interstitial lung disease,and Raynaud phenomenon.We present a case of a unique combination of deep morphea in a patient with anti-U1RNP myositis.Case presentation:A 64-year-old male with 5-year history of proximal muscle weakness,polyarthritis,Raynaud phenomenon,and dyspnea on multiple immunosuppressives presented with localized infiltrated,tight,and hyperpigmented plaques over the posterior thighs and mid-to-lower back developing over the last 2 years and limiting his movement.Autoimmune workup revealed a positive ANA,anti-U1RNP antibody,anti-Jo1 antibody,and anti-Ro52 antibody.Further workup showed restrictive lung disease,kidney disease,and arthritis.Patient was diagnosed with anti-U1RNP myositis.Skin biopsy of the back lesion showed deep morphea.Discussion:Association of deep morphea with anti-U1RNP myositis is not described prior in the literature.Treatment of morphea is challenging since the patient is already on immunosuppressive medications.The patient failed methotrexate prior and is currently on Mycophenolate mofetil and Deflazacort which are reported as potential treatment for morphea.Therefore,physical therapy plus topical Tacrolimus were suggested as an initial measure to preserve the range of motion of his posterior thighs and back.This is a case of progressive deep morphea developing in a patient with a unique autoimmune profile on immunosuppressive drugs.Conclusion:Anti-U1RNP myositis is a challenging diagnosis and should be always thought of in patients with positive anti-U1RNP,myositis,interstitial lung disease,arthritis,kidney disease,and Raynaud phenomenon.Moreover,deep morphea treatment in immunosuppressed patients is challenging and different measures should be considered.展开更多
Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder that damages multiple organs simultaneously and is associated with chronic inflammation, in which the signs of systemic sclerosis, systemic...Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder that damages multiple organs simultaneously and is associated with chronic inflammation, in which the signs of systemic sclerosis, systemic lupus erythematosus, and polymyositis can co-occur. Its distinctive feature and the basis for the diagnosis of MCTD is the presence of an antibody against the uridine-rich RNP of the cell nucleus (Anti-U1-RNP). It has been observed that intercurrent infections can trigger autoimmune diseases, however, the fact that viral infections—such as SARS-CoV-2—induce them is currently poorly understood. The present study raises the etiological role of the SARS-CoV-2 virus in the development of the disease. Authors describe the case of a 38-year-old patient in good general condition, who was diagnosed with mixed connective tissue disease three months after COVID-19 infection.展开更多
文摘Introduction:Morphea is an inflammatory skin disease characterized by skin thickening due to increased collagen deposition in the dermis or subcutaneous tissues.Anti-U1RNP myositis is a newly described entity characterized by myositis,arthritis,interstitial lung disease,and Raynaud phenomenon.We present a case of a unique combination of deep morphea in a patient with anti-U1RNP myositis.Case presentation:A 64-year-old male with 5-year history of proximal muscle weakness,polyarthritis,Raynaud phenomenon,and dyspnea on multiple immunosuppressives presented with localized infiltrated,tight,and hyperpigmented plaques over the posterior thighs and mid-to-lower back developing over the last 2 years and limiting his movement.Autoimmune workup revealed a positive ANA,anti-U1RNP antibody,anti-Jo1 antibody,and anti-Ro52 antibody.Further workup showed restrictive lung disease,kidney disease,and arthritis.Patient was diagnosed with anti-U1RNP myositis.Skin biopsy of the back lesion showed deep morphea.Discussion:Association of deep morphea with anti-U1RNP myositis is not described prior in the literature.Treatment of morphea is challenging since the patient is already on immunosuppressive medications.The patient failed methotrexate prior and is currently on Mycophenolate mofetil and Deflazacort which are reported as potential treatment for morphea.Therefore,physical therapy plus topical Tacrolimus were suggested as an initial measure to preserve the range of motion of his posterior thighs and back.This is a case of progressive deep morphea developing in a patient with a unique autoimmune profile on immunosuppressive drugs.Conclusion:Anti-U1RNP myositis is a challenging diagnosis and should be always thought of in patients with positive anti-U1RNP,myositis,interstitial lung disease,arthritis,kidney disease,and Raynaud phenomenon.Moreover,deep morphea treatment in immunosuppressed patients is challenging and different measures should be considered.
文摘Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disorder that damages multiple organs simultaneously and is associated with chronic inflammation, in which the signs of systemic sclerosis, systemic lupus erythematosus, and polymyositis can co-occur. Its distinctive feature and the basis for the diagnosis of MCTD is the presence of an antibody against the uridine-rich RNP of the cell nucleus (Anti-U1-RNP). It has been observed that intercurrent infections can trigger autoimmune diseases, however, the fact that viral infections—such as SARS-CoV-2—induce them is currently poorly understood. The present study raises the etiological role of the SARS-CoV-2 virus in the development of the disease. Authors describe the case of a 38-year-old patient in good general condition, who was diagnosed with mixed connective tissue disease three months after COVID-19 infection.
