Lymphomatoid papulosis and anaplastic large cell lymphomas of the skin

It is now generally accepted that primary CD30+ cutaneous lymphomas comprise a clinical and morphologic spectrum in which a clear distinction between lymphomatoid papulosis (LyP) and lymphoma cannot always be made. Management varies from observation in patients who have relatively asymptomatic, spontaneously remitting disease (as in LyP) to multiagent chemotherapy regimens with or without autologous stem cell transplantation in patients whose disease has spread to involve extracutaneous sites other than regional lymph nodes (as in disseminated CD30+ lymphoma). Choosing an appropriate management strategy requires correlation of the patient's clinical history (including symptoms) with physical exam and pathologic findings. The importance of clinicopathologic correlation cannot be overemphasized, because lesions with clinically "benign" behavior may appear "malignant" by pathology, and failure to interpret pathologic findings in accordance with the patient's clinical history and physical exam can result in unnecessary, overly aggressive, and potentially harmful treatments. This review highlights integration of clinical and pathologic features of these primary cutaneous CD30+ lymphoproliferative disorders.