Chronic active Epstein–Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome

Chronic active Epstein–Barr virus infection with cutaneous lymphoproliferation:... Dear Editor,Patients with chronic active Epstein–Barr virus (EBV) infections (CAEBV) present with cutaneous manifestations including hydroa vacciniforme‐like eruptions. Haemophagocytic lymphohistiocytosis (HLH), a fatal complication, can occur in severe cases of CAEBV. We retrieved 25 cases of CAEBV treated in our hospital and histopathologically characterized the skin lesions of three of those cases, including one case with haemophagocytosis. This is a novel report of CAEBV with haemophagocytosis in the skin.Patient No. 1: A 29‐year‐old male who had been suffering from multiple indurated erythematous plaques on his hands and lower limbs for 15 months (Fig. a, b). Laboratory investigations revealed a decreased number of platelets: 4.3 × 104/μL (normal: 13–30 × 104/μL). The titres of anti‐EBV antibodies were as follows: viral capsid antigen (VCA) IgG ×640; VCA IgM less than ×10; EBV nuclear antigen ×80. The EBV genome load in peripheral blood (PB) was 7.7 × 105 copies/μg DNA. His EBV‐infected cells were CD56‐positive, and the diagnosis of CAEBV was made according to Kimura et al. Histopathological examination showed atypical lymphoid cells in the dermis and subcutaneous adipose tissue (Fig. c,d). Infiltrates consisted of atypical lymphoid cells (Fig. e). Immunohistochemical examination showed positive reactions for CD3, CD56 (Fig. f), TIA‐1 (Fig. g) and granzyme B. Atypical lymphoid cells were positive for EBV‐encoded small RNA http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of the European Academy of Dermatology & Venereology Wiley

Chronic active Epstein–Barr virus infection with cutaneous lymphoproliferation: haemophagocytosis in the skin and haemophagocytic syndrome

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Publisher
Wiley
Copyright
Copyright © 2018 European Academy of Dermatology and Venereology
ISSN
0926-9959
eISSN
1468-3083
D.O.I.
10.1111/jdv.14640
Publisher site
See Article on Publisher Site

Abstract

Dear Editor,Patients with chronic active Epstein–Barr virus (EBV) infections (CAEBV) present with cutaneous manifestations including hydroa vacciniforme‐like eruptions. Haemophagocytic lymphohistiocytosis (HLH), a fatal complication, can occur in severe cases of CAEBV. We retrieved 25 cases of CAEBV treated in our hospital and histopathologically characterized the skin lesions of three of those cases, including one case with haemophagocytosis. This is a novel report of CAEBV with haemophagocytosis in the skin.Patient No. 1: A 29‐year‐old male who had been suffering from multiple indurated erythematous plaques on his hands and lower limbs for 15 months (Fig. a, b). Laboratory investigations revealed a decreased number of platelets: 4.3 × 104/μL (normal: 13–30 × 104/μL). The titres of anti‐EBV antibodies were as follows: viral capsid antigen (VCA) IgG ×640; VCA IgM less than ×10; EBV nuclear antigen ×80. The EBV genome load in peripheral blood (PB) was 7.7 × 105 copies/μg DNA. His EBV‐infected cells were CD56‐positive, and the diagnosis of CAEBV was made according to Kimura et al. Histopathological examination showed atypical lymphoid cells in the dermis and subcutaneous adipose tissue (Fig. c,d). Infiltrates consisted of atypical lymphoid cells (Fig. e). Immunohistochemical examination showed positive reactions for CD3, CD56 (Fig. f), TIA‐1 (Fig. g) and granzyme B. Atypical lymphoid cells were positive for EBV‐encoded small RNA

Journal

Journal of the European Academy of Dermatology & VenereologyWiley

Published: Jan 1, 2018

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