
Sézary syndrome: Report of a rare case with perioral manifestation and review of the literature
2022; Elsevier BV; Volume: 134; Linguagem: Inglês
10.1016/j.oraloncology.2022.106134
ISSN1879-0593
AutoresCarla Isabelly Rodrigues‐Fernandes, Pablo Agustín Vargas, Iara Gonçalves Aquino, Márcio Ajudarte Lopes, Alan Roger Santos‐Silva,
Tópico(s)Nail Diseases and Treatments
ResumoThis report aimed to describe a rare case of Sézary syndrome (SS) diagnosed in an Oral Medicine service. A 54-year-old female presented a generalized pruritus and erythema of the skin of 2 years in duration, which had been treated with antihistamines, corticosteroids, and hydrating creams, without resolution. Extra-oral examination showed a painful lymphadenopathy on the right supraclavicular region. Ultrasound-guided fine-needle aspirationbiopsy did not detect any abnormalities. The patient's skin was remarkably dry and thickened, with erythroderma, fissures, and ulcerations. The perioral region exhibited extreme peeling and angular cheilitis. Immunophenotyping of peripheral blood revealed proliferation of undifferentiated T-cells and a massive proportion of TCD4+ cells relative to TCD8+ cells. PET/CT examination demonstrated multiple lymphadenopathies, and bone marrow biopsy was negative for neoplastic cell infiltration. A diagnosis of SS was established, and the patient is currently being treated with UVB phototherapy, methotrexate, doxepin, and folic acid, with mostly complete regression of signs and symptoms.
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