Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases.

Abstract

Hydroa vacciniforme (HV)-like lymphoma (HVL) is a rare and aggressive cutaneous T-cell lymphoma occurring mainly in children in Latin America and Asia. Chronic latent Epstein-Barr virus infection has been associated with both HV and HVL. We sought to evaluate the clinical presentation and histopathology of this rare cutaneous T-cell lymphoma. We reviewed the clinical, morphologic, and immunophenotypical features in 12 cases of HVL from Bolivia. All 12 patients had skin lesions in both sun-exposed and nonsun-exposed areas, including edema, blistering, ulceration, and scarring, with a slowly progressive relapsing course. All 12 patients presented with systemic symptoms and showed a characteristic swelling of the nose and lips, and periorbital edema. Eight patients died an average of 5.3 months after initial diagnosis. Four patients remained alive with persistent disease. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity. The immunophenotype showed a cytotoxic T-cell (CD8(+)) profile. All cases were associated with Epstein-Barr virus infection and differed clinically from other forms of cutaneous T-cell lymphoma. Only a limited number of cases were studied. This study confirms that HVL is a highly aggressive lymphoma, although some patients have a more indolent, chronic course.