mycosis fungoides


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  • Clinical

    • most common cutaneous T lymphoma (50%)
    • site: skin

  • Morphology

    • cerebriform nuclei
    • epidermis and upper dermis infiltrate
    • vacuolar interface dermatitis with lymphocyte in nearly every vacuole
    • Pautrier microabscesses in epidermis
    • progression
      • early: superficial infiltrate (band or lichenoid)
      • later: more diffuse dermal infiltrate
    • variants
      • folliculotropic: involve hair follicles, spares epidermis
      • Pagetoid reticulosis: Can be either CD4 or CD8 positive. CD30+/-.
      • Granulomatous slack skin: lax skin in major skin folds, CD4+ granulomatous infiltrate

  • Stains

    • positive: CD2, CD3, CD4, CD5, TCRB
    • negative: CD8 (rare CD8+ cases)

  • Staging

    • Clinical
      • I: skin confined
        • IA: < 10%
        • IB: > 10%
      • II: node involvement or tumor
        • IIA: node involvment
        • IIB: tumor
      • III: erythroderma
      • IV: > 1000/uL Sezary cells in PB, node effacement (N3), or visceral involvement
    • Nodal
      • N1: none to rare atypical cerebriform cells
      • N2: atypical cerebriform cells, scattered to aggregates
      • N3: node effacement, partial to complete
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