Sézary Syndrome: Different Erythroderma Morphological Features with Proposal for a Clinical Score System
2022
Gabriele Roccuzzo | Silvia Giordano | Gianluca Avallone | Marco Rubatto | Silvia Canonico | Ada Funaro | Erika Ortolan | Rebecca Senetta | Paolo Fava | Maria Teresa Fierro | Simone Ribero | Pietro Quaglino
Sézary syndrome is a rare subtype of cutaneous T-cell lymphoma characterized by erythroderma, peripheral lymphadenopathies, and circulating atypical cerebriform T-cells. To date, no definite staging system has been developed for these patients. In this retrospective analysis of the archive of the Dermatological Clinic of the University of Turin, Italy, erythrodermic SS patients were classified according to clinical records and photographs into three main presentations: erythematous, infiltrated, or melanodermic. The pattern of erythroderma was found to be associated with disease outcome, as better survivals were recorded in patients with erythematous and infiltrative erythroderma. Patients in the melanodermic group, though less represented in our investigation, seemed to show a worse trend in survival. According to this preliminary evidence, a new prognostic classification, with a revised score specific for Sézary syndrome patients, can be proposed to usefully integrate the current staging system. The correlation displayed in our research will be hopefully confirmed by prospective studies with larger cohorts, with the aim of identifying significant prognostic features in this subset of cutaneous T-cell lymphoma patients.
Show more [+] Less [-]Bibliographic information
This bibliographic record has been provided by Directory of Open Access Journals
