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How I treat primary cutaneous CD30+ lymphoproliferative disorders

Michi M. Shinohara and Andrei Shustov

Abstract

The primary cutaneous CD30+ lymphoproliferative disorders are a family of extranodal lymphoid neoplasms that arise from mature post-thymic T-cells and localize to the skin. Current classification systems recognize lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma, and overlap cases. In the majority of patients, the prognosis of primary cutaneous CD30+ lymphoproliferative disorders is excellent, however, relapses are common and complete cures are rare. Skin directed and systemic therapies are utilized as monotherapy or in combination to achieve the best disease control and minimize overall toxicity. We discuss three distinct presentations of primary cutaneous CD30+ lymphoproliferative disorder, and present recommendations for a multidisciplinary team approach to diagnosis, evaluation, and management of these conditions in keeping with existing consensus guidelines.

  • Submitted March 26, 2019.
  • Revision received May 18, 2019.
  • Accepted May 14, 2019.