Sézary Syndrome without Erythroderma: A Case Report

Abstract

Background: Sézary syndrome (SS) is an aggressive leukaemic form of cutaneous T-cell lymphoma characterised by erythroderma, lymphadenopathy and circulating malignant T cells. The absence of erythroderma at presentation may complicate recognition. Case presentation: A 76-year-old woman with insulin-treated type 2 diabetes mellitus and hypertension presented with a one-year history of generalised pruritus unresponsive to topical corticosteroids, oral antihistamines and emollients. Examination showed infiltrated erythematous plaques involving the face and limbs, hypopigmented macules on the trunk, palmoplantar keratoderma and scalp, axillary and pubic hair loss, without erythroderma. Skin histopathology revealed an epidermotropic infiltrate of atypical lymphocytes with cerebriform nuclei. Immunohistochemistry demonstrated CD3, CD4, CD5 and CD7 expression and a predominance of CD4-positive lymphocytes. Peripheral blood testing showed lymphocytosis, Sézary cells accounting for 17% of lymphocytes (1,700/µL) and a CD4/CD8 ratio of 95. Investigations supported the diagnosis of SS staged T2aN1M0B2 (IVA1). CHOEP chemotherapy was followed by marked improvement in skin lesions and pruritus. Diffuse erythroderma developed with relapse two years after treatment completion. Conclusion: This case illustrates how SS may initially present without erythroderma despite suggestive cutaneous and haematological findings. Persistent pruritus with palmoplantar keratoderma, alopecia or substantial blood involvement should prompt consideration of SS and integrated clinical, histopathological, immunophenotypic and haematological assessment. Continued follow-up is important because the cutaneous phenotype may change over time.