Sézary Syndrome without Erythroderma in the Early Stage of the Disease

Abstract

A 79-year-old male suffered from intense pruritus for about 10 months. Topical corticosteroid applications provided little relief. Physical examination showed faint red papules located symmetrically on the neck, trunk, and limbs without erythroderma or desquamation. On skin biopsy specimen, atypical lymphocytes infiltrated densely into the upper dermis with epidermotropism, and Pautrier microabscesses were present. Peripheral blood tests showed Sézary cell count ＞1,000/μL. Flow cytometry revealed infiltration of tumor cells into lymph nodes and bone marrow. The patient was diagnosed as Sézary syndrome (SS). Sézary syndrome is a cutaneous T-cell lymphoma defined by erythroderma, lymphadenopathy, and circulating atypical T cells with poor prognosis. In our case, the symptoms were intense pruritus and inconspicuous eruption, without erythroderma at the first visit. Only 25.5% of SS patients present with erythroderma from the first visit, and 5.7% do not eventually develop erythroderma. It is reported that 86% of SS cases without erythroderma have strong pruritus. Although the mechanism of erythroderma has not been clarified, immune abnormalities and partial deletion of skin-homing molecules in Sézary cells may contribute to the absence of erythroderma. Dermatologists should be aware of non-erythrodermic variants of SS. Skin Research, 21 : 267-271, 2022