title: Chronic active EBV infection with features of granulomatosis with polyangiitis.
abstract: Herein, we report the case of a 13-year-old boy with multiple recurrent ulcers on his legs. He developed severe sinusitis at 10 years of age and had significant weight loss (6 kg) in the 2 months prior to admission. Histology of tissue biopsied from the ulcer indicated small vessel vasculitis and granulomatous inflammation. Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. Further pathology, however, indicated Epstein-Barr virus (EBV)-encoded RNA (EBER) in most lymphocytes in the same sample. The EBER-positive lymphocytes were mainly CD4-positive T cells. The EBV-DNA load in the peripheral blood was also abnormally increased (1.0 × 10(4) copies/μg DNA). Thus, the diagnosis was established as chronic active EBV infection (CAEBV). This case illustrates the necessity of careful differential diagnosis of CAEBV owing to its clinical resemblance and pathological overlap with GPA.
authors.affiliation: Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Shimomura
authors.affiliation: Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Morishita
authors.affiliation: Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Takaaki
authors.initials: T
authors.lastname: Meguro
authors.affiliation: Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Seto
authors.affiliation: Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Kimura
authors.affiliation: Department of Pathology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Minoru
authors.initials: M
authors.lastname: Hamazaki
authors.affiliation: Department of Otorhinolaryngology, Shizuoka Children's Hospital, Shizuoka, Japan.
authors.forename: Ayako
authors.initials: A
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, Shizuoka Medical Center, Shizuoka, Japan.
authors.forename: Yuka
authors.initials: Y
authors.lastname: Sugiyama
authors.affiliation: Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002908:Chronic Disease; D004279:DNA, Viral / Q000032:analysis*; D003937:Diagnosis, Differential; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis* / Q000821:virology; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis*; D004854:Herpesvirus 4, Human / Q000235:genetics*; D006801:Humans; D007871:Leg Ulcer / Q000175:diagnosis / Q000209:etiology*; D008214:Lymphocytes / Q000473:pathology*; D008297:Male
chemical_list: D004279:DNA, Viral
keywords: anti-neutrophil cytoplasmic antibody; chronic active Epstein-Barr virus infection; granulomatosis with polyangiitis; sinusitis; ulcer