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Clinical and Histological Characteristics of Mycosis Fungoides and Sézary Syndrome: A Retrospective, Single-centre Study of 43 Patients from Eastern Denmark

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  • Pia Rude Nielsen
  • Jens Ole Eriksen
  • Ulrike Wehkamp
  • Lise Maria Lindahl
  • Robert Gniadecki
  • Hanne Fogh
  • Susanne Fabricius
  • Michael Bzorek
  • Niels Ødum
  • Lise Mette Gjerdrum
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Diagnosis of mycosis fungoides and Sézary syndrome can be very challenging. Clinical and histopathological data for patients with mycosis fungoides and Sézary syndrome in Denmark are limited. A retrospective study was performed in Region Zealand, Denmark from 1990 to 2016. A total of 43 patients with mycosis fungoides or Sézary syndrome were identified during the period. At the time of diagnosis the patients' mean age was 64.3 years and 74.5% had early-stage (≤IIA) disease. The mean time from onset of skin disease to diagnosis was 4.4 years. Surprisingly, 43% progressed to a higher disease stage, and risk of disease progression was higher for stage IB than IA (p = 0.01). All cases displayed some degree of epidermotropism and the infiltrates consisted of pleomorphic lymphocytes with a T-helper (CD4+/CD8-) phenotype. This study describes, for the first time, all aspects of clinical and histopathological findings in patients with mycosis fungoides and Sézary syndrome in a well-characterized Danish cohort.

Original languageEnglish
JournalActa Dermato-Venereologica
Volume99
Issue number13
Pages (from-to)1231-1236
Number of pages6
ISSN0001-5555
DOIs
Publication statusPublished - 1 Dec 2019

ID: 58955669