Diagnostic Approach to Adult Erythroderma: A Rare Case of Sezary Syndrome.

Bibliographic Details
Title: Diagnostic Approach to Adult Erythroderma: A Rare Case of Sezary Syndrome.
Authors: Savitri, Quri Meihaerani1, Rettobyan, Fitria2, Astari, Linda2, Rahmatika, Amira Suryani, Diansyah, Muhammad Noor3, Amrita, Putu Niken Ayu3, Romadhon, Pradana Zaky3, Bintoro, Sipirianus Ugroseno Yudho3,4, Ashariati, Ami3, Savitri, Merlyna3 merlyna.savitri@gmail.com
Source: Iranian Journal of Pathology. Fall2024, Vol. 19 Issue 4, p460-466. 7p.
Subject Terms: *CUTANEOUS T-cell lymphoma, *SYMPTOMS, *HOSPITAL admission & discharge, *HEPATITIS B, *SKIN biopsy, *DYSTROPHY
Abstract: Background & Objective: Sezary Syndrome is an uncommon leukemic variant of Cutaneous T-cell Lymphoma (CTCL), comprising only 5% of all CTCL cases. The rarity of this syndrome emphasizes the critical need to comprehend its distinct clinical presentation, diagnosis, and treatment. Case Presentation: A 51-year-old man was admitted with itchy, persistent, and extensive erythematous patches, ulcers, lumps, lymphadenopathy, alopecia, and nail dystrophy that had been present for eight months. Laboratory findings showed elevated LDH and β2-microglobulin. Peripheral blood smear analysis confirmed the presence of Sezary cells, while imaging revealed multiple lymph node enlargements. Skin biopsy and immunohistochemistry suggested cutaneous T-cell lymphoma (CTCL), while immunophenotyping verified a diagnosis of Sezary syndrome . The patient underwent fluid therapy, systemic antibiotics, topical antibiotics, phototherapy, and chemotherapy. Tenofovir was given due to the hepatitis B co-infection. Despite the improvement when discharged from the hospital, the patient's health eventually deteriorated, which led to death at home. Conclusion: This patient presented with Sezary Syndrome, exhibiting atypical dermatologic manifestations that must be differentiated from other causes of erythroderma. This case highlights the importance of a comprehensive diagnostic approach, including clinical evaluation, laboratory tests, imaging, and biopsies. Sezary Syndrome is an inherently aggressive malignancy, characterized by a poor response to treatment and a low 5-year survival rate. [ABSTRACT FROM AUTHOR]
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Database: Academic Search Complete
More Details
ISSN:17355303
DOI:10.30699/IJP.2024.2022723.3258
Published in:Iranian Journal of Pathology
Language:English