[Skin involvement as the presenting feature of primary systemic anaplastic lymphoma kinase-negative CD30+ anaplastic lymphoma].

We report the case of a 66-year-old man who consulted with a 3-week history of a rapidly growing asymptomatic lesion on his left cheek that for the previous week had prevented him from opening the eye on that side (Fig. 1). Two years earlier at another center the patient had been diagnosed with a primary systemic CD30 anaplastic large-cell lymphoma (ALCL), with skin lesions in the same area as the lesion described above and ipsilateral cervical lymph-node involvement at the time of diagnosis. The patient had received chemotherapy (CHOP: cyclophosphamide, doxorubicin, vincristine, and prednisone) and external radiotherapy, with complete remission of the skin lesions and lymph-node involvement. Examination revealed a 15× 12 cm plaque affecting the malar, infraocular, and preauricular regions on the left side, extending down to the angle of the mandible. The lesion had poorly defined borders and was formed by the confluence of multiple tumors (Fig. 1). Histology revealed marked epidermal spongiosis that gave rise to scattered intraepidermal vesicles and intense edema of the papillary dermis (Fig. 2A). A dense lymphocytic proliferation affected the rest of the dermis and extended into the subcutaneous tissue (Fig. 2A). The neoplastic infiltrate was formed by large pleomorphic cells with abundant clear cytoplasm. The nuclei were kidney-shaped or oval with unevenly distributed chromatin and prominent nucleoli (Fig. 2B). Immunohistochemistry was positive for CD3 and CD30 (Fig. 2C) in 95% of neoplastic cells. CD5 and CD15 expression was focal, with the markers detected in 30% and 20% of the cells, respectively. Ki-67 antigen was positive in 80% to 90% of the neoplastic infiltrate. In addition, 30% of cells were positive for CD4 and 15% were positive for CD8, while anaplastic lymphoma kinase (ALK) 1, CD56, and epithelial membrane antigen were negative. Genetic study of the T-cell receptor showed monoclonal reordering. Further tests included an analysis of T-cell populations and immunoglobulin levels, a computed tomography of the head, neck, abdomen and pelvis, and a bone-marrow biopsy. All the results were normal or negative. These findings led to a diagnosis of cutaneous recurrence of ALK-negative CD30 anaplastic large-cell lymphoma, and chemotherapy was initiated. The patient showed partial remission after 2 complete cycles of CHOP. One month