Leukocytoclastic vasculitis as early manifestation of Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly

• Published in: The American journal of dermatopathology Vol. 34; no. 3; pp. 330 - 334
• Main Authors: Zoroquiain, Pablo, González, Sergio, Molgó, Montserrat, Rodríguez, Alejandra, Valbuena, José R
• Format: Journal Article
• Language: English
• Published: United States 01-05-2012
• Subjects: Aged; Antibodies, Monoclonal, Murine-Derived - administration & dosage; Antibodies, Monoclonal, Murine-Derived - therapeutic use; Antineoplastic Combined Chemotherapy Protocols - administration & dosage; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Cyclophosphamide - administration & dosage; Cyclophosphamide - therapeutic use; Diagnosis, Differential; Doxorubicin - administration & dosage; Doxorubicin - therapeutic use; Drug Therapy, Combination; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - diagnosis; Epstein-Barr Virus Infections - drug therapy; Fatal Outcome; Glucocorticoids - therapeutic use; Herpesvirus 4, Human - isolation & purification; Humans; Immunosuppressive Agents - therapeutic use; Lymphoma, Large B-Cell, Diffuse - diagnosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - virology; Male; Methylprednisolone - therapeutic use; Prednisone - administration & dosage; Prednisone - therapeutic use; Sepsis; Vasculitis, Leukocytoclastic, Cutaneous - diagnosis; Vasculitis, Leukocytoclastic, Cutaneous - drug therapy; Vasculitis, Leukocytoclastic, Cutaneous - virology; Vincristine - administration & dosage; Vincristine - therapeutic use
• ISSN: 0193-1091; 1533-0311
• DOI: 10.1097/DAD.0b013e3182318dd9

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.