Overview of skin lymphomas WHO-EORTC classification (2018)

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: -

Mycosis fungoides, Sézary syndrome (SS), Adult T-cell leukemia/lymphoma (HTLV+), Primary cutaneous CD30+ lymphoproliferative disorders, Primary cutaneous anaplastic large cell lymphoma (PCALCL), Lymphomatoid papulosis (LyP), Subcutaneous panniculitis-like T-cell lymphoma (SPTCL), Extranodal NK/T-cell lymphoma, Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma (provisional), Cutaneous γ/δ T-cell lymphoma (provisional), Primary cutaneous small-medium pleomorphic T-cell lymphoma (provisional), Primary cutaneous follicular B-cell lymphoma (PCFCL), Primary cutaneous marginal zone B-cell lymphoma (PCMZL), Primary cutaneous diffuse large B-cell lymphoma, Primary cutaneous intravascular large B-cell lymphoma.

Current WHO-EORTC classification (2018)

  • Cutaneous T cell lymphoma:
    • Mycosis fungoides (MF)
    • MF variants
      • Folliculotropic MF
      • Pagetoid reticulosis
      • Granulmatous slack skin
      • Sezary Syndrome
      • Adult T Cell leukemia / lymphoma
    • Prinary cutaneous CD30+ LPDs
      • C-ALCL
      • Lymphomatoid papulosis
      • Subcutaneous panniculitis-like T cell lymphoma
      • Extranodal NK/T cell lymphoma, nasal type
      • Chronic active EBV infection
    • Primary cutaneous T cell lymphoma, rare subtypes
      • Primary cutaneous gamma/delta T cell lymphoma
      • CD8+ AECTCL (provisional)
      • Primary cutaneous CD4+ small/medium T cell lymphoproliferative disorder (provisional)
      • Primary cutaneous acral CD8+ T cell lymphoma (provisional)
      • Primary cutaneous peripheral T cell lymphoma, NOS
  • Cutaneous B cell lymphoma:
    • PCMZL
    • PCFCL
    • PCDLBLC, LT
    • EBV+ mucocutaneous ulcer (provisional)
    • Intravascular large B cell lymphoma