What are the histologic findings of primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, characterized by a proliferation of epidermotropic CD8+ cytotoxic T cells, has an acanthotic or atrophic epidermis, necrotic keratinocytes, ulceration, and variable spongiosis, sometimes with blister formation. [38, 69] Epidermotropism tends to be marked, ranging from a linear distribution to a pagetoid pattern throughout the epidermis.

This disorder has a beta-F1+, CD3+, CD8+, granzymes-B+, perforin+, TIA-1+, CD45RA+, CD45RO, CD2-, CD4-, CD5-, CD7+/- phenotype. [53, 69] It is usually associated with EBV seronegativity. The lymphoma cells show clonal TCR gene rearrangements, although specific genetic abnormalities have not been described.


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