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Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases
Yanghe Liu1, Cuiling Ma1, Gang Wang1
1Department of Dermatology, Xijing Hospital, Fourth Military Medical University, No. 15 Changlexi Road, Xian 710032, China.
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.
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