A High Programmed Cell Death Protein 1 Hormone Receptor Score on Skin Biopsy is Associated with Sézary Syndrome Diagnosis: A Study of 91 Patients with Erythroderma

Authors

  • Camille Luherne Dermatology Department, CHU de Bordeaux, F-33000 Bordeaux, France
  • Sarah Menguy Pathology Department, CHU Bordeaux, F-33600 Pessac, France
  • Thomas Ferte Pole de Sante Publique, Unité de Soutien Méthodologique à la Recherche Clinique et Epidémiologique, CHU de Bordeaux, F-33000 Bordeaux, France
  • Marie Beylot-Barry Dermatology Department, CHU de Bordeaux, F-33000 Bordeaux, France
  • Julien Seneschal Dermatology Department, CHU de Bordeaux, F-33000 Bordeaux, France
  • Brigitte Milpied Dermatology Department, CHU de Bordeaux, F-33000 Bordeaux, France
  • Jean-Philippe Vial Hematology Biology Department, CHU Bordeaux, F- 33600 Pessac, France
  • Audrey Gros Tumor Biology and Tumor Bank Department, CHU Bordeaux, F-33600 Pessac, France
  • Samuel Amintas Tumor Biology and Tumor Bank Department, CHU Bordeaux, F-33600 Pessac, France
  • Béatrice Vergier Pathology Department, CHU Bordeaux, F-33600 Pessac, France
  • Anne Pham-Ledard Dermatology Department, CHU de Bordeaux, Bordeaux, France

DOI:

https://doi.org/10.2340/actadv.v102.1062

Keywords:

Sézary syndrome, programmed-death 1, erythrodermic mycosis fungoides, erythrodermic psoriasis, erythrodermic eczema, erythrodermic drug-induced erythroderma, programmed cell death protein 1

Abstract

Erythroderma is challenging to diagnose. The aim of this single-centre retrospective study was to identify factors that can be used to improve the diagnosis of erythroderma. Among 91 patients with erythroderma, 21 were diagnosed with eczema, 17 with psoriasis, 20 with drug-induced erythroderma, 13 with erythrodermic mycosis fungoides and 20 with Sézary syndrome. Nail alterations, ear involvement, and severe scaling were significantly associated with psoriasis (p = 0.044). Fever and hypereosinophilia were associated with drug-induced erythroderma. Expression of programmed cell death protein 1 was observed in all skin biopsies. However, with Sézary syndrome, programmed cell death protein 1 expression was significantly higher than with other aetiologies. A programmed cell death protein 1 hormone receptor score (H-score) >50 was associated with Sézary syndrome (p < 0.001, sensitivity 75%, specificity 92%) as well as CXCL13 expression (p < 0.044). CD7 loss was more frequent with erythrodermic mycosis fungoides and Sézary syndrome (p = 0.022). This study reports the importance of programmed cell death protein 1 expression for the differential diagnosis of Sézary syndrome and other aetiologies, including erythrodermic mycosis fungoides.

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Published

2022-09-06

How to Cite

Luherne, C., Menguy, S., Ferte, T., Beylot-Barry, M., Seneschal, J., Milpied, B., Vial, J.-P., Gros, A., Amintas, S., Vergier, B., & Pham-Ledard, A. (2022). A High Programmed Cell Death Protein 1 Hormone Receptor Score on Skin Biopsy is Associated with Sézary Syndrome Diagnosis: A Study of 91 Patients with Erythroderma. Acta Dermato-Venereologica, 102, adv00773. https://doi.org/10.2340/actadv.v102.1062

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