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2018, Number 3

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Rev Cent Dermatol Pascua 2018; 27 (3)

Sézary syndrome. Communication of a case

Domínguez-Gómez MA, Ramos-Garibay JA, Vidal-Flores AA

Language: Spanish
References: 17
Page: 96-103
PDF size: 324.83 Kb.


ABSTRACT

Sézary syndrome (SS) is a leukemic variant of cutaneous T-cell lymphoma, clinically characterized by the presence of erythrodermia, and at the blood level by atypical T cells (known as Sézary cells) with or without lymphadenopathy. In this article we report the case of a 58-year-old female patient, with erythroderma that started as pruritic erythematous plaques in forearms. She also had involvement of cutaneous annexes (diffuse alopecia, onychodystrophy of the 20 nails), ocular (xerophthalmia, ectropion) and oral (xerostomy, gingivitis). Biopsy was performed, and the histopathological report was compatible with a lymphoproliferative process. Peripheral blood smear reported: large lymphocytes with cleaved nuclei, 26% blasts; immunohistochemistry: CD45+, CD45Ro+, CD4+; the tomography revealed adenomegalies. With the above, the diagnosis of Sézary syndrome was established. Chemotherapy and electron beam treatment started, showing partial improvement. Palliative treatment was added with phototherapy and general skin measures with favorable evolution.


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