Cutaneous T-cell lymphoma: Differential diagnostics of erythrodermic conditions

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Abstract


Erythroderma is a condition damaging at least 80–90 % of the skin surface, characterized by erythema and peeling. This condition may be a manifestation of various diseases of the skin and internal organs, including malignant lymphoproliferative disorders. Sézary syndrome and the erythrodermic variant of mycosis fungoides are aggressive forms of cutaneous T-cell skin lymphoma, the diagnostics of which is challenging due to the similar clinical pictures of these diseases with benign dermatoses. This article presents two clinical cases of erythroderma in the setting of cutaneous T-cell lymphoma. An analysis of the anamnestic data, the clinical picture and the results of laboratory examinations in patients suffering from this condition allowed the diagnosis of Sézary syndrome to be confirmed. The presented cases reflect the importance of identifying the causes of erythroderma.

Conflict of interest: the authors state that there is no potential conflict of interest requiring disclosure in this article.


About the authors

A. E. Karamova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Russian Federation Arfenya E. Karamova — Cand. Sci. (Med.), Head of the Department of Dermatology

V. V. Chikin

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Russian Federation Vadim V. Chikin — Dr. Sci. (Med.), Senior Researcher, Department of Dermatology

L. F. Znamenskaya

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Email: fake@neicon.ru

Russian Federation Lyudmila F. Znamenskaya — Dr. Sci. (Med.), Leading Researcher, Department of Dermatology

A. A. Vorontsova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Health of the Russian Federation

Author for correspondence.
Email: vorontsova@cnikvi.ru

Russian Federation Anastasia A. Vorontsova — Junior Researcher, Department of Dermatology

References

  1. Burns T. et al. Rook’s textbook of dermatology. 8th ed. Oxford: Willey-Blackwell, 2010. Vol. 4. P. 4432.
  2. Wolf K. et al. Fitzpatrick’s dermatology in general medicine. 7th ed. New York: McGraw-Hill, 2007. P. 225–232.
  3. Sigurdsson V., Steegmans P. H., van Vloten W. A. The incidence of erythroderma: a survey among all dermatologists in The Netherlands. J Am Acad Dermatol. 2001;45(5):675–678.
  4. Rothe M. J., Bernstein M. L., Grand-Kels J. M. Life-threatening erythroderma: diagnosing and treating “the red” man. Clin Dermatol. 2005;23:206–217.
  5. Sarkar R., Sharma R. C., Koranne R. V. et al. Erythroderma in children — a clinico-etiologic study. J Dermatol. 1999;26:507–511.
  6. Каламкарян А. А., Авербах Е. В., Забанова Е. В., Персина И. С. Клинические особенности злокачественных лимфом кожи. Вестник дерматологии и венерологии. 1989;(12):8–12.
  7. Родионов А. Н. Эритодермическая лимфома кожи: Учебное пособие. Л., 1989. 67 с.
  8. Сейкетова О. Ж., Джумамуратова Д. И. Результаты клинического обследования больных псориазом за 10 лет. Вестник дерматологии и венерологии. 1970;(1):19–21.
  9. Pileri A., Pellegrini C., Agostinelli C. et al. Erythroderma and non-Hodgkin T-cell lymphoma: what else, apart from mycosis fungoides and Sézary syndrome? Eur J Dermatol. 2017;27(1):49–53.
  10. Gros A., Laharanne E., Vergier M. et al. TP53 alterations in primary and secondary Sézary syndrome: A diagnostic tool for the assessment of malignancy in patients with erythroderma. PLoS One. 2017;12(3):e0173171.
  11. Martinez X. U., Di Raimondo C., Abdulla F. R., Zain J., Rosen S. T., Querfeld C. et al. Leukaemic variants of cutaneous T-cell lymphoma: Erythrodermic mycosis fungoides and Sézary syndrome. Best Pract Res Clin Haematol. 2019 Sep;32(3):239–252.
  12. Willemze R., Cerroni L., Kempf W., Berti E., Facchetti F., Swerdlow S. H. et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019 Jan;133(16):1703–1714.
  13. Vonderheid E. C., Bernengo M. G., Burg G., Duvic M., Heald P., Laroche L. et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the international society for cutaneous lymphomas. J Am Acad Dermatol. 2002 Jan;46(1):95–106.
  14. Olsen E., Vonderheid E., Pimpinelli N., Willemze R., Kim Y., Knobler R. et al. Revisions to the staging and classification of mycosis fungoides and sezary syndrome: a proposal of the international society for cutaneous lymphomas (ISCL) and the cutaneous lymphoma task force of the European organization of Research and treatment of cancer (EORTC). Blood. 2007 Sep 15;110(6):1713–1722.
  15. Olsen E.A., Rook A.H., Zic J., Kim Y., Porcu P., Querfeld C. et al. Sezary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. 2011 Feb;64(2):352–404.
  16. Белоусова И. Э., Казаков Д. В., Криволапов Ю. А. Современные подходы к диагностике и лечению первичных лимфом кожи на основе новой ВОЗ/ EORTC — классификации. Т-клеточные лимфомы кожи. Архив патологии. 2007;69(5):11–17.
  17. Burg G., Kempf W. Cutaneous Lymphomas (Basic and Clinical Dermatology). Informa Health-care, 2005. 592 p.
  18. Mistry N., Gupta A., Alavi A., Sibbald R. G. A review of the diagnosis and management of erythroderma (generalized red skin). Adv Skin Wound Care. 2015;28(5):228–236.
  19. Klemke C. D., Booken N., Weiss C. et al. Histopathological and immunophenotypicalcriteria for the diagnosis of Sézary syndrome indifferentiation from other erythrodermic skindiseases: a European Organisation for Re-search and Treatment of Cancer (EORTC)Cutaneous Lymphoma Task Force Study of97 cases. Br J Dermatol. 2015;173(1):93–105.
  20. Olsen E. A. Evaluation, diagnosis, and staging of cutaneous lymphoma. Dermatol Clin. 2015 Oct;33(4):643–654.

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