Hydroavacciniforme-like lymphoproliferative disorder with long-term follow-up

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated cutaneous lymphoproliferative disorder with heterogeneous clinical behavior. We report a 15-year-old boy with a two-year history of recurrent facial edema and papulovesicular lesions initially misdiagnosed as angioedema. Skin biopsy revealed EBV-positive cytotoxic T‑cell infiltration with TCRβ gene rearrangement, confirming HVLPD. The patient responded to corticosteroids, interferon-α, acyclovir, and intravenous immunoglobulin (IVIG), achieving symptom resolution and EBV-DNA normalization. One year later, he relapsed with fever, worsening facial swelling, and new lesions; hemophagocytic lymphohistiocytosis was excluded. Re-induction with glucocorticoids, interferon-α, IVIG, and empirical antibiotics led to improvement. This case underscores the relapsing nature of HVLPD and the importance of long-term EBV-DNA monitoring and individualized immunomodulatory management.