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A woman in her 70s presented with a 3-month history of episodic fever and painless cutaneous lesions on her lower limbs. The patient otherwise felt well and denied malaise, weight loss, or any other symptoms. She did not have any known benign inflammatory conditions. Physical examination showed indurated plaques with multiple telangiectasias (Figure, A and B) on almost all the surface of the legs. Except for slightly elevated C-reactive protein levels, the results of other routine blood and urine tests were unremarkable. A skin biopsy specimen was obtained for histopathology examination (Figure, C and D).
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C. Intravascular large B-cell lymphoma
Histopathologic evaluation revealed large round cells with scant cytoplasm and prominent nucleoli proliferating within blood vessels. Immunohistochemical analysis showed positivity for CD20, B-cell lymphoma (Bcl) 2, Bcl-6, cellular MYC, and multiple myeloma 1. The Ki-67 staining index was about 90%. The endothelial cells of the involved vessels expressed CD34 and CD31 and were negative for D2-40. Positron emission tomography scan results revealed no lymphadenopathy or metastases. No evidence of bone marrow involvement was found. The diagnosis of intravascular large B-cell lymphoma (IVLBCL) cutaneous variant was made. The patient was treated with 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) with a complete response. At her last follow-up, 1 year after treatment, she continued to be in remission.
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Corresponding Author: Claudia Sarró-Fuente, MD, Department of Dermatology, Hospital Universitario Fundación Alcorcón, Calle Budapest, 1, 28922 Alcorcón, Madrid, Spain (email@example.com).
Published Online: August 25, 2021. doi:10.1001/jamadermatol.2021.3051
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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