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A case of hydroa vacciniforme-like lymphoproliferative disorder with indolent clinical course
( Sang Hyung Lee ) , ( Hyun Ji Kang ) , ( Woo Jin Lee ) , ( Chong Hyun Won ) , ( Sung Eun Chang ) , ( Mi Woo Lee ) , ( Jee Ho Choi )
UCI I410-ECN-0102-2017-510-000102312
This article is 4 pages or less.

Primary cutaneous hydroa vacciniforme(HV)-like lymphoma is a rare, Epstein-Barr virus-associated cutaneous neoplasm occurring mainly in childhood and usually associated with poor prognosis. This report presents a case of primary cutaneous HV-like lymphoma with unusual indolent clinical course and favorable prognosis during the follow-up periods of 6 months. She presented with 20-year history of erythema, papules, and tiny ulcerations without progression on the face and 2-month history of solitary pinkish mass on the left thigh. Skin biopsy revealed spongiotic vesicle or necrosis with dense infiltration of small- to medium sized atypical lymphoid cells in the dermis. The lymphoid cells were immunoreactive to CD3, CD4 and CD8, mixed, and revealed the negativity for CD56. The Epstein-Barr virus genomes were also found in skin biopsy. By genetic analysis, she showed βF-1/T-cell receptor gene clonal rearrangement. Fortunately, solitary mass of the left thigh was spontaneously resolved before treatment. The patients underwent minocycline and NSAIDs 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.

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