primary cutaneous gamma delta T cell lymphoma

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  • Clinical

    • skin lesions: plaques, ulcerated nodules, tumors
    • most frequently extremities
    • can have hemophagocytic syndrome
    • rapidly progressive
  • Histology

    • infiltrate: epidermotropic, dermal and subQ
    • cytology: medium-large cells
    • apoptosis, necrosis, angioinvasion
    • lymphocytes rimming fat cells
  • Stains

    • positive: TCR gamma delta, CD2, CD3, CD56, cytotoxic (granzyme B, TIA1, perforin)
    • negative: CD5, CD4, CD8, beta F1
  • Ddx

    • subcutaneous panniculitis like T cell lymphoma (TCR gamma delta-, beta F1+, fat only)