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. 2023 Jul;50(7):e198-e205.
doi: 10.1111/1346-8138.16842. Epub 2023 May 30.

Diagnostic and disease severity determination criteria for hydroa vacciniforme lymphoproliferative disorders and severe mosquito bite allergy

Yoji Hirai  1 , Hideo Asada  2 , Toshihisa Hamada  3 , Jun-Ichi Kawada  4 , Hiroshi Kimura  5 , Ayako Arai  6 , Koichi Ohshima  7 , Shouichi Ohga  8 , Keiji Iwatsuki  1 ; Committee for Guidelines for the Management of Chronic Active EBV Disease and Related Disorders (the MHLW Research Team in Japan)
Affiliations

Diagnostic and disease severity determination criteria for hydroa vacciniforme lymphoproliferative disorders and severe mosquito bite allergy

Yoji Hirai et al. J Dermatol. 2023 Jul.

Abstract

Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) and severe mosquito bite allergy (SMBA) are both cutaneous forms of Epstein-Barr virus (EBV)-associated T/natural killer (NK) cell LPDs and are closely related to chronic active EBV disease (CAEBV) and EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). HV-LPD is further divided into classic HV, a benign subtype mediated by EBV-positive γδT cells, and systemic HV, another life-threatening subtype mainly associated with EBV-positive αβT or γδT cells. The vast majority of patients with SMBA have increased numbers of EBV-infected NK cells in the blood. Clinical symptoms of HV-LPD and SMBA often overlap in the same patient and may progress to more serious disease conditions equivalent to the systemic form of CAEBV. To define the disease spectrum of HV-LPD and SMBA, we propose the diagnostic criteria and the determination criteria for disease severity. The proposed diagnostic criteria are consistent with those for CAEBV and EBV-HLH in the guidelines for the management for CAEBV and related disorders 2023.

Keywords: Epstein-Barr virus; diagnostic criteria; disease spectrum; hydroa vacciniforme lymphoproliferative disorder; severe mosquito bite allergy.

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References

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