Lymphoma & related disorders
Mature T/NK cell disorders
Cutaneous / soft tissue involvement
Primary cutaneous CD4+ small / medium

Topic Completed: 1 August 2011

Minor changes: 18 January 2021

Copyright: 2001-2021,, Inc.

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

Dragos C. Luca, M.D.
Page views in 2020: 1,204
Page views in 2021 to date: 91
Cite this page: Luca D. Primary cutaneous CD4+ small / medium. website. Accessed January 22nd, 2021.
Definition / general
  • Cutaneous nodular proliferation of pleomorphic T-lymphocytes of undetermined significance
  • Rare, 2% of all cutaneous T cell lymphomas
  • Solitary plaque or nodule on face, neck, upper trunk
  • Lower extremity involvement is rare
Clinical features
  • Usually asymptomatic; only clinical feature is a solitary skin lesion
  • Rarely large tumors or multiple skin lesions
Case reports
Treatment and prognosis:
  • Favorable prognosis, 80% 5 year survival
  • Solitary skin lesions have an excellent prognosis (surgical excision or radiotherapy), multiple / large lesions may be more aggressive
Postulated normal counterpart
  • Skin homing CD4+ T cell
Gross description
  • Solitary plaque or nodule (more favorable) or multiple or large lesions (more aggressive)
Gross images

Images hosted on other servers:

Various images

Microscopic (histologic) description
  • Dense, diffuse or nodular dermal infiltrate with tendency to subcutaneous involvement
  • May have focal epidermotropism but if more than that, consider mycosis fungoides
  • Predominantly small / medium sized pleomorphic T cells sometimes with a small proportion (< 30%) of large pleomorphic cells
  • May have admixed reactive lymphocytes, histiocytes, eosinophils, plasma cells
Positive stains
Negative stains
  • CD8, CD30, cytotoxic markers, loss of a pan-T cell marker, EBV
Molecular / cytogenetics description
  • Clonal rearrangements of the TCR genes
  • No specific cytogenetic abnormality
Differential diagnosis
  • Reactive lymphoid infiltrate of the skin: also called pseudolymphoma or cutaneous lymphoid hyperplasia; no clonal TCR gene rearrangements, no loss of a pan-T cell marker
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