A Fatal Case of Severe Leg Edema and Necrotic Ulcers in a 14-Year-Old Boy.

A 14-year-old boy presented with edema, ulcers, tenderness, and progressive functional limitation of both legs, first diagnosed as Henoch-Schonlein vasculitis. Then, he underwent one inguinal lymph node excision and two skin biopsies which reported an angiocentric lymphoproliferative process, EBER (Epstein-Barr virus-encoded small RNA) positive, consistent with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD); after eight weeks, his face presented with edema and ulcers, characteristic of the original patients described with HVLPD. The patient's parents refused treatment and took him back home, and he died a few months later. Our case study highlights an atypical localization of the disease, as it initially presented in the lower extremities rather than the face, posing a diagnostic challenge that was ultimately resolved through biopsy.