A Case of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Presenting As Orogenital Ulcerations.

Abstract

Corresponding Author Yang Wang Department of Dermatology and Venerology, Peking University First Hospital, No.8 Xishiku Street, Xicheng District, Beijing 100034, China Tel: +86-10-83572350, Fax: +86-10-66551216, E-mail: yangwang_dr@bjmu.edu.cn https://orcid.org/0000-0001-7805-2861 Dear Editor: Hydroa vacciniforme (HV)-like lymphoproliferative disease (LPD) is associated with chronic active Epstein-Barr virus (CAEBV) infection. It ranges from classic and severe/systemic HV to HV-like lymphoma (HVLL). Cutaneous manifestations include vesiculopapules, bullae, ulcers, and facial swelling. HVLL usually has a long clinical course with spontaneous resolution but may progress to more severe and lifethreatening conditions. We report a rare case of HV-like LPD that evolved from classic HV to orogenital ulcerations. A 17-year-old Chinese boy presented with a 2-month history of painful orogenital swelling and ulcerative lesions without constitutional symptoms. He was diagnosed with classic HV at age 2 years, based on the typical papulovesicular eruptions on his face and photosensitivity. The symptoms spontaneously resolved 2 years before without recurrence, leaving facial varioliform scars (Fig. 1A). Physical examination revealed marked swelling on the lower lip and massive erosions with ulcerations on the scrotum (Fig. 1A, B). No lymphadenopathy was observed. A skin biopsy from the lower lip showed diffuse infiltrates of atypical lymphoid cells throughout the dermis, with remarkable epidermotropism (Fig. 2A). Immunohistochemical analysis revealed CD3+++, CD4+++, Granzyme B++, and TIA1+++ infiltrating lymphocytes, consistent with