Abstract:
BACKGROUND:Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement. OBJECTIVE:We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma. METHODS:Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied. RESULTS:Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred. LIMITATIONS:Retrospective design and small sample size are limitations. CONCLUSION:Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Henn A,Michel L,Fite C,Deschamps L,Ortonne N,Ingen-Housz-Oro S,Marinho E,Beylot-Barry M,Bagot M,Laroche L,Crickx B,Maubec Edoi
10.1016/j.jaad.2014.11.015subject
Has Abstractpub_date
2015-06-01 00:00:00pages
1003-9.e1issue
6eissn
0190-9622issn
1097-6787pii
S0190-9622(14)02199-9journal_volume
72pub_type
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