Overview of skin lymphomas WHO-EORTC classification (2018)

Last Updated: 2023-07-07

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

ICD11: -

Synonym (s)

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.

Classification

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