Hydroa vacciniforme-like lymphoproliferative disorder with peculiar presentation

Abstract This case is an atypical presentation of hydroa vacciniforme-like lymphoproliferative disorder (HVLPD). A 14-year-old boy presented with edema, ulcers, tenderness and progressive functional limitation of both legs, he underwent one inguinal lymph node excision and two skin biopsies which reported an angiocentric lymphoproliferative process EBER positive consistent with HVLPD. After 8 weeks his face presented with edema and ulcers, characteristic of the original patients described with HVLPD. Patient´s parents refused treatment and took him back home, he died a few months later. We highlight not only the atypical and the usual bad prognosis of this disease but also that taking an early biopsia in an unclear clinical condition can make the diagnosis and impact on patients life.