Lymphoma and plasma cell neoplasms
T / NK cell disorders
Primary cutaneous CD4+ small / medium T cell lymphoma

Author: Dragos Luca, M.D. (see Authors page)

Revised: 29 March 2017, last major update August 2011

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PubMed search: primary cutaneous CD4+ small / medium T cell lymphoma

Cite this page: Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma . PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBCD8epidermotropic.html. Accessed August 20th, 2017.
Definition / general
Terminology
  • Similar (? identical) to the generalized pagetoid reticulosis of the Ketron-Goodman type
Epidemiology
  • Rare, < 1% of all cutaneous TCL’s
  • Mainly adults
Sites
  • Generalized skin lesion in most patients
Clinical features
  • Localized or disseminated papules, nodules and tumors with central ulceration and necrosis, or superficial hyperkeratotic patches and plaques
  • Extracutaneous dissemination possible: lung, testis, CNS, oral mucosa (lymph nodes often spared)
Case reports
Prognostic factors
Treatment and prognosis:
  • Aggressive clinical course
  • Median survival 32 months
Postulated normal counterpart
  • Skin homing CD8+ cytotoxic T cells of αβ type
Gross description
  • Localized or disseminated papules, nodules and tumors with central ulceration and necrosis, or superficial hyperkeratotic patches and plaques
Gross images

Images hosted on other servers:

Image (Fig. 5A)

Microscopic (histologic) description
  • Very variable appearance
  • Lichenoid pattern with marked pagetoid epidermotropism and subepidermal edema
  • Deep nodular infiltrates
  • Epidermal acanthosis or atrophy with necrosis, ulceration, blister formation; invasion and destruction of skin adnexal structures
  • Small, medium or large tumor cells with pleomorphic or blastic nuclei; angiocentricity and angioinvasion may be present
Microscopic (histologic) images

Images hosted on other servers:

Image (Fig. 5B)

Positive stains
Negative stains
Molecular / cytogenetics description
  • Clonal TCR gene rearrangements
  • No specific cytogenetic abnormalities
Differential diagnosis