A case of bullous Sézary syndrome

Abstract

Sézary syndrome is a rare leukemic subtype of cutaneous T cell lymphoma that is characterized by erythroderma, lymphadenopathy, and malignant T cells in the peripheral blood. Poor prognostic factors of Sézary syndrome include advanced disease stage, older age at onset, and large cell transformation. Presentation with bullous lesions, though rare, has been reported in a few patients. We present an elderly woman with bullous Sézary syndrome who presented with a two-month history of progressive rash. Upon admission, the patient had pruritic, erythematous, edematous plaques with overlying flaccid bullae and erosions involving the scalp, neck, torso, and extremities. Despite treatment, the patient died two months after presentation. Although rare, bullous lesions associated with Sézary syndrome may indicate poor prognosis.