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

Publish Year: 1403
Type: Journal paper
Language: English
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JR_IJP-19-4_013

Index date: 2 February 2025

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

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

Quri Savitri

Department of Internal Medicine, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Fitria Rettobyan

Department of Internal Medicine, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Linda Astari

Department of Dermatology and Venereology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Amira Rahmatika

Department of Dermatology and Venereology, Faculty of Medicine, Airlangga University, Surabaya, Indonesia

Muhammad Diansyah

Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Putu Amrita

Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Pradana Romadhon

Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Sipirianus Bintoro

Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Ami Ashariati

Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

Merlyna Savitri

Hematology and Medical Oncology Division, Department of Internal Medicine, Dr. Soetomo General Teaching Hospital, Surabaya, Indonesia

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