Rapidly progressive adult-onset hydroa vacciniforme-like lymphoproliferative disorder associated with Epstein-Barr virus: a case report

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated T- or natural killer (NK)-cell lymphoproliferative disease that classically affects children. Adult-onset disease is uncommon and may follow an aggressive systemic course. We report a case of systemic adult-onset HVLPD in a 63-year-old woman who initially presented symptoms concerning for an autoimmune process. Initial biopsies demonstrated thrombotic vasculopathy and granulomatous dermatitis without malignant features. Despite transient improvement with immunosuppressive therapy, she later developed rapidly progressive disease and repeat biopsy confirmed and EBV-associated clonal T-cell lymphoproliferative process consistent with systemic HVLPD. Staging studies demonstrated widespread nodal and extranodal disease. The patient received CHOEP chemotherapy followed by nivolumab after early progression; however, the disease remained refractory, and she ultimately elected for hospice care. This case highlights the diagnostic difficulty of adult-onset HVLPD when early manifestations mimic inflammatory or vasculopathic disease.