Sézary syndrome without erythroderma

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.

Keywords: Sézary cells; Sézary syndrome; chronic pruritus; erythroderma; invisible dermatosis; leukemic cutaneous T-cell lymphoma; nonerythrodermic; prognosis.