Erythroderma and non-Hodgkin T-cell lymphoma: what else, apart from Mycosis Fungoides and Sézary syndrome?

Eur J Dermatol. 2017 Feb 1;27(1):49-53. doi: 10.1684/ejd.2016.2915.

Abstract

Background: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature.

Objectives: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin.

Materials & methods: Three cases of PTCL-NOS were investigated; all male, with a mean and median age of 55 and 51 years, respectively.

Results: All patients underwent aggressive chemotherapeutic protocols with only transient improvement of the disease, and died within two years of follow-up.

Conclusion: Dermatologists should be reminded that erythroderma and lymph node enlargement do not represent an exclusive paradigm for erythrodermic cutaneous T-cell lymphoma, and that these features can be due to a systemic lymphoma that should be considered in the differential diagnosis.

Keywords: PTCL-NOS; erythroderma; lymph node enlargement; peripheral T-cell lymphomas not otherwise specified; systemic lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Deoxycytidine / analogs & derivatives
  • Deoxycytidine / therapeutic use
  • Dermatitis, Exfoliative / etiology*
  • Diagnosis, Differential
  • Doxorubicin / therapeutic use
  • Etoposide / therapeutic use
  • Fatal Outcome
  • Humans
  • Lymphadenopathy / etiology
  • Lymphoma, T-Cell, Peripheral / complications*
  • Lymphoma, T-Cell, Peripheral / diagnosis*
  • Lymphoma, T-Cell, Peripheral / drug therapy
  • Lymphoma, T-Cell, Peripheral / pathology
  • Male
  • Middle Aged
  • Mycosis Fungoides / diagnosis*
  • Organoplatinum Compounds / therapeutic use
  • Prednisolone / therapeutic use
  • Prednisone / therapeutic use
  • Sezary Syndrome / diagnosis*
  • Vincristine / therapeutic use

Substances

  • Organoplatinum Compounds
  • Deoxycytidine
  • Vincristine
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide
  • Prednisolone
  • Prednisone

Supplementary concepts

  • CHOEP protocol
  • CHOP protocol
  • gemcitabine-oxaliplatin regimen