Abstract
Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (PCSMP-TLPD) is a provisional entity with uncertain malignant potential according to the latest revision of the WHO classification for lymphoid neoplasms. We conducted a systematic literature review of all previously reported cases of PCSMP-TLPD to highlight their typical and atypical features. The main features of PCSMP-TLPD and its possible clinicopathologic overlap with similar disorders are also discussed. It is hoped that this review will provide a useful outline of this condition and the most important differential diagnoses. Finally, we recommend a rigorous consensus among cutaneous lymphoma experts in drafting diagnostic criteria and the best case definition.
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