Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases.,Journal of the American Academy of Dermatology

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Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases.
Journal of the American Academy of Dermatology ( IF 12.8 ) Pub Date : 2019-01-14 , DOI: 10.1016/j.jaad.2019.01.011
Yanghe Liu ₁ , Cuiling Ma ₁ , Gang Wang ₁ , Lei Wang ₁

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BACKGROUND Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease that is usually found in East Asians and Latin Americans. OBJECTIVE To report the characteristics of HVLLPD in Chinese patients. METHODS Retrospective analysis of patients with HVLLPD from a single institute. RESULTS A total of 41 patients were enrolled. All patients presented with papulovesicular lesions, mainly distributed on sun-exposed areas, with 26 patients showing systemic symptoms. Follow-up data were available for 20 patients, 16 patients were alive, and 4 patients died. Of the 4 deceased patients, 3 had taken a serum EBV DNA test that showed high viral loads. These 3 patients also received chemotherapy. Histopathology was characterized by dense proliferation of lymphocytes in the dermis. Angiotropism or angiodestruction was found in the majority of patients, whereas prominent cellular polymorphism was noticed in only 4 patients. All patients were positive for CD3, TIA1 cytotoxic granule associated RNA binding protein, and EBV-encoded RNA in situ hybridization. LIMITATIONS This was a retrospective study. CONCLUSIONS HVLLPD in Chinese patients showed indolent behavior in the majority of cases, which differed from the characteristics of HVLLPD in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of their disease evolving into aggressive disease. Chemotherapy should not be considered as first-line treatment for most Chinese patients.

更新日期：2019-07-12

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