Hydroa vacciniforme-like lymphoproliferative disorder: Clinicopathologic study of 41 cases

Publication date: Available online 14 January 2019

Source: Journal of the American Academy of Dermatology

Author(s): Yanghe Liu, Cuiling Ma, Gang Wang, Lei Wang

Abstract

Background

Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease usually found in East Asians and Latin Americans.

Objective

To report the characteristics of HVLLPD in Chinese patients.

Methods

Retrospective analysis of HVLLPD patients from a single institute.

Results

Forty-one patients were enrolled. All cases presented with papulovesicular lesions, mainly distributed on sun-exposed areas. Twenty-six cases showed systemic symptoms. Follow-up data was available in 20 cases, 16 patients were alive, and 4 patients died. Among the 4 deceased patients, 3 had serum EBV DNA test and 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 majority cases, while prominent cellular polymorphism was noticed in only 4 cases. All cases were positive for CD3, TIA-1, 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 HVLLPD characteristics in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of evolving into aggressive disease. Chemotherapy should not be considered as first line treatment for most Chinese patients.

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