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: Masaki Shimomura, Hideaki Morishita, Takaaki Meguro, Shiro Seto, Mitsuaki Kimura, Minoru Hamazaki, Ayako Hashimoto, Yuka Sugiyama, Hiroshi Kimura
• Journal: Pediatrics international : official journal of the Japan Pediatric Society (Pediatr Int) Vol. 58 Issue 7 Pg. 639-42 (Jul 2016) ISSN: 1442-200X [Electronic] Australia
• PMID: 26952849 (Publication Type: Case Reports)
• Copyright: © 2016 Japan Pediatric Society.

Chemical References

• DNA, Viral

Topics

• Adolescent
• Biopsy
• Chronic Disease
• DNA, Viral (analysis)
• Diagnosis, Differential
• Epstein-Barr Virus Infections (complications, diagnosis, virology)
• Granulomatosis with Polyangiitis (complications, diagnosis)
• Herpesvirus 4, Human (genetics)
• Humans
• Leg Ulcer (diagnosis, etiology)
• Lymphocytes (pathology)
• Male