Epstein-Barr Virus Associated Lymphoproliferative Lesion Presenting as a Hydroa Vacciniforme-like Eruption

Cho, Kwang Hyun; Li, Kap Sok; Kim, Yon Kyung; Jeon, Yoon Kyung; Kim, Chul Woo; Lee, Suk Kyeong; Moon, Sang Eun; Sohn, Sook Ja

Abstract

BACKGROUND
There have been several reports of patients with a severe hydroa vacciniforme (HV)-like eruption from Asia and Latin America. The cutaneous lesions are present in both sun-exposed and nonexposed areas in these patients unlike true HV. Several patients have died of malignant hematologic malignancies. The latent Epstein-Barr virus (EBV) infection has been detected in the skin lesions of the patients. OBJECTIVE: To describe clinical, histological, immunohistochemical, and molecular pathological features of the patients with EBV associated lymphoproliferative lesion presenting as a HV-like eruption. METHODS: The clinical, histological, and immunohistochemical features of 16 patients were reviewed. The presence of T-cell receptor (TCR)-gamma gene rearrangement was investigated using polymerase chain reaction (PCR) technique. Photoprovocation by repetitive UVA exposure was performed in five patients. In situ hybridization was performed to detect mRNA for EBV in the lesional skin biopsy specimen, lymph node biopsy specimen, mucosal biopsy specimen of stomach, and the skin biopsy specimen of photo-provoked site. PCR was performed to detect DNA for EBV in the skin biopsy specimens of 6 patients and peripheral mononuclear cells of 2 patients. RESULTS: The severity of the skin lesion and the clinical course varied among the patients. Skin biopsy specimens obtained from a papule or a vesicle showed perivascular and periadnexal infiltrate of lymphoid cells with T-cell phenotype. However, clonal TCR-gamma gene rearrangement was not detected in all 8 patients. Papules or vesicles were induced by repetitive UVA exposure in 5 patients. A latent EBV infection was demonstrated in all the tested samples, such as lesional skin, lymph node, gastric mucosa, peripheral blood mononuclear cells, and the photo-provoked lesion. CONCLUSION: EBV associated lymphoproliferative lesion presenting as a HV-like eruption is a novel disease that is not related to classic HV. Repetitive irradiation of UVA can induce the skin lesion in some patients with EBV associated lymphoproliferative lesion presenting as a HV-like eruption.