Primary cutaneous hydroa vacciniforme-like lymphoma with indolent clinical course: Report of two cases and review of literature

Primary cutaneous hydroa vacciniforme (HV)-like lymphoma is a rare, Epstein-Barr virus-associated cutaneous neoplasm characterized by photosensitive papulovesicular eruption and usually associated with poor prognosis. This report presents 2 cases of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 2 and 3 years, respectively. Both patients presented with erythema, papulovesicles, scars, ulcerations, and edema on the face and extremities. Skin biopsies revealed epidermal vesicle with small- to medium-sized atypical lymphoid cells infiltrating in the dermis and subcutis. The lymphoid cells were strongly immunoreactive to CD8 and CD56. The Epstein-Barr virus genomes were also found in both skin biopsies. By genetic analysis, one of patients showed T-cell receptor-γ gene clonal rearrangement. The patients underwent glucocorticoid treatment and obtained remarkable clinical improvement with regression of skin lesions. No sign of recurrence and extracutaneous manifestation was found during the period of follow-up. A long-term follow-up is suggested to be performed to inspect the progression for this tumor.