Abstract

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a CD30+ lymphoproliferative disease with generally favorable outcomes and infrequent extracutaneous spread, usually limited to local lymph nodes. However, there may be extensive histological overlap with more aggressive CD30+ lymphomas, such as large cell transformation of mycosis fungoides or secondary skin involvement by ALK-negative anaplastic large cell lymphoma. Definitive diagnosis as a result relies heavily on clinicopathologic correlation. We report on a 26-year-old female with pcALCL, initially treated locally now with recent progressive cutaneous disease started on systemic chemotherapy, who presented for management with presumed septic shock.  Dermatology was consulted for chronic lower extremity wounds. Skin examination revealed scattered yellow ulcers with an erythematous rim on the trunk, axilla, and left lower extremity with a larger, ulcerated plaque with an exophytic black necrotic core on the right lower extremity of which was biopsied. Histopathology demonstrated an ulcerated epidermis and diffuse sheets of large, anaplastic, CD30-positive and ALK-1-negative T-cells. Imaging revealed stage IV disease with innumerable, intensely FDG-avid subcutaneous, intramuscular, and visceral foci, but paucity/absence of lymph node involvement. The patient’s condition deteriorated, and she expired during this hospitalization. This case will review the clinicopathologic findings of pcALCL vs. other typically more aggressive lymphomas, emphasize the importance of clinicopathologic correlation in differentiating between CD30+ lymphoproliferative disorders, and highlight the extremely rare phenomenon of systemic intramuscular and visceral disseminated disease occurring in pcALCL.