We report a 14-year-old Indian boy who presented with a history of weight loss, fever, facial edema, and a relapsing papulovesicular eruption on the face and limbs for 1 year. Histopathology of the skin showed dense lymphoid infiltrate from dermis to subcutaneous fat. Immunohistochemistry of this lymphoid infiltrate was CD3+, CD8+, CD56+, CD57+, Granzyme B+, TIA+, and Epstein Barr virus LMP1+. The histopathology and immunohistochemistry were consistent with the diagnosis of hydroa vacciniforme-like T-cell lymphoma. The child responded remarkably to oral steroids but relapsed on tapering doses. CHOP (Cyclophosphamide, Adriamycin, Vincristine, and Prednisolone) chemotherapy was initiated in view of systemic involvement to which he showed some response, however, the disease relapsed again. He then had a rapidly progressive disease and ultimately succumbed to his illness. This is the first case of hydroa vacciniforme-like T-cell lymphoma being reported from this subcontinent.
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