Sézary syndrome mimicking Steven-Johnson syndrome: A case report

Sézary syndrome is a rare and subtype of non-Hodgkin T cell lymphomas. It typically presents as papules, macules, nodules, or ulcers, frequently accompanied by lymphadenopathy and systemic symptoms. This study describes uncommon clinical and diagnostic features to distinguish Sézary syndrome from serious skin and mucous membrane disorders through a detailed case report and comprehensive literature review.

A 70-year-old female patient was diagnosed with progressive Sézary syndrome associated with extensive cutaneous scaly and ulcerative lesions after 5 cycles of anthracycline-based chemotherapy which is initially misleading to Steven-Johnson syndrome (SJS). SJS was clinically excluded due to no mucosal involvement. She received a single agent of steroid as a lymphoma salvage treatment due to poor performance and nutrition status. Facial and left arm skin biopsy revealed T lymphoma cells infiltrating the upper dermis. Unfortunately, she died due to progressive lymphoma disease and upper gastrointestinal bleeding.

Unlike mycosis fungoides, Sézary syndrome involves more than bathing suit areas of skin and it may present with scaly, ulceration, or even hemorrhagic bullae and Sézary cells in peripheral blood. Sometimes, it's hard to differentiate from SJS or toxic epidermal necrolysis (TEN). Mucosa involvement is a crucial feature shown in patients with SJS/TEN.