Abstract
Extensive necrotizing vasculitis (ENV) is a rare paraneoplastic phenomenon, and the majority of cases reported are associated with hematolymphoid neoplasms. Histologically, most cases of ENV represent leukocytoclastic vasculitis (LCV). Here we report the clinicopahological features of a 68-year-old man with ENV associated to a Epstein Barr virus-positive diffuse large B-cell lymphoma (EBV+DLBCL) of the elderly, a newly recognized lymphoproliferative disorder, most likely representing a paraneoplastic manifestation. The patient was treated with standard chemotherapy regimen for malignant lymphoma. Due to the extensive involvement of the extremities by ENV, surgical debridement was not feasible and a novel therapy based on CHITOSAN apposits was initiated with overall good response and subsequent re-epithelization of the skin lesions. The patient died of sepsis secondary to a Pseudomona pneumonia 17 months after diagnosis.
MeSH terms
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Aged
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Antibodies, Monoclonal, Murine-Derived / administration & dosage
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Antibodies, Monoclonal, Murine-Derived / therapeutic use
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Cyclophosphamide / administration & dosage
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Cyclophosphamide / therapeutic use
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Diagnosis, Differential
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Doxorubicin / administration & dosage
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Doxorubicin / therapeutic use
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Drug Therapy, Combination
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Epstein-Barr Virus Infections / complications
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Epstein-Barr Virus Infections / diagnosis*
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Epstein-Barr Virus Infections / drug therapy
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Fatal Outcome
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Glucocorticoids / therapeutic use
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Herpesvirus 4, Human / isolation & purification*
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Humans
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Immunosuppressive Agents / therapeutic use
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Lymphoma, Large B-Cell, Diffuse / diagnosis*
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Lymphoma, Large B-Cell, Diffuse / drug therapy
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Lymphoma, Large B-Cell, Diffuse / virology
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Male
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Methylprednisolone / therapeutic use
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Prednisone / administration & dosage
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Prednisone / therapeutic use
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Rituximab
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Sepsis
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Vasculitis, Leukocytoclastic, Cutaneous / diagnosis*
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Vasculitis, Leukocytoclastic, Cutaneous / drug therapy
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Vasculitis, Leukocytoclastic, Cutaneous / virology
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Vincristine / administration & dosage
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Vincristine / therapeutic use
Substances
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Antibodies, Monoclonal, Murine-Derived
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Glucocorticoids
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Immunosuppressive Agents
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R-CHOP protocol
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Rituximab
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Vincristine
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Doxorubicin
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Cyclophosphamide
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Prednisone
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Methylprednisolone
Supplementary concepts
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Erythema elevatum diutinum