Epidermotropic precursor T-cell lymphoma with highly aggressive clinical behavior simulating localized pagetoid reticulosis.

We describe a 43-year-old patient with a solitary, eczematous plaque on his right nipple that had developed during the previous 6 weeks. Histopathology revealed a superficial band-like infiltrate of medium to large-sized pleomorphic lymphocytes with striking epidermotropism. The tumor cells expressed a T-phenotype (CD3+, CD20-) and were CD30-, CD4-, and CD8-negative. A diagnosis of localized pagetoid reticulosis (Woringer-Kolopp disease) with possible large cell transformation was proposed. A cervical lymph node excised 2 months later showed features of a highly aggressive blastoid precursor T-cell lymphoma. Reevaluation of the skin lesion and of a tonsil specimen previously interpreted as benign hyperplasia showed features consistent with those observed in the lymph node. The disease was rapidly progressive, and the patient died 15 months after the first skin biopsy. This case represents a unique cutaneous presentation of aggressive precursor lymphoma, showing the protean nature of lymphoproliferative disorders and the overlapping clinical and histopathologic features of different entities.