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Primary cutaneous T cell lymphomas, rare subtypes

Rein Willemze, MD
Section Editor
John A Zic, MD
Deputy Editor
Rosamaria Corona, MD, DSc


The term primary cutaneous T cell lymphoma (CTCL) refers to T cell lymphomas that present primarily in the skin without evidence of extracutaneous disease at the time of diagnosis. The group of classical CTCLs (mycosis fungoides, variants of mycosis fungoides, and Sézary's syndrome) and the group of primary cutaneous CD30+ lymphoproliferative disorders (anaplastic large cell lymphoma and lymphomatoid papulosis) encompass the most common forms of CTCLs, accounting for approximately 90 percent of CTCLs in the Western world [1]. However, different distributions have been observed in other parts of the world [2,3].

Other types of CTCLs recognized in the 2005 World Health Organization (WHO)-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas [1] and in the 2016 revision of the WHO classification of tumors of hematopoietic and lymphoid tissue [4] include uncommon entities such as: subcutaneous panniculitis-like T cell lymphoma; extranodal natural killer/T cell lymphoma, nasal type; primary cutaneous peripheral T cell lymphoma (PTCL), not otherwise specified; and rare subtypes of PTCL [1,4].

This topic will focus on rare subtypes of primary cutaneous PTCL, which include:

Primary cutaneous gamma-delta T cell lymphoma

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T cell lymphoma

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Literature review current through: Oct 2017. | This topic last updated: Nov 21, 2016.
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