How is primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma characterized?

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 is a clinically aggressive, (sometimes) disseminated disease that presents with eruptive papules, nodules, and tumors with central ulceration. This entity can also present with superficial patches and/or plaques. Affected patients have typically been treated with anthracycline-based systemic chemotherapy.

Histologically, epidermotropism with invasion and destruction of adnexal skin structures and angiocentricity with angioinvasion can be seen. The malignant cells are CD3- and CD8-positive and contain cytotoxic proteins. Clonal T-cell gene rearrangement is seen. EBV tests are typically negative in primary aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma.


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