A 69-year-old male presented to the Infectious Disease Service with erythematous scaly lesions persisting for five years, which had evolved into diffuse exfoliative erythroderma and multiple disseminated scaly plaques in the three weeks preceding admission. Physical examination revealed infiltrated facial appearance, serous blistering, ulcerated lesions in the oral and genital mucosa, infiltrated plaques on the face and auricular pavilion, crusted scaly plaques on the anterior/posterior trunk and upper and lower limbs with lesion exulceration, intense pruritus, fever, arthralgia, and diffuse lymphadenopathy w(Figure 1). Laboratory findings at admission included LDH 672 U/L, PCR 152.91 mg/dL, 75,000 leukocytes/µL, atypical lymphocytes, and convoluted nucleus lymphomatous cells suggestive of Sézary cells (10-15% in peripheral blood smears) (Figure 2). The clinical course was unfavorable, with a worsening state after 10 days, preceding specific interventions.
Disseminated ulcerations and erythroderma on the chest, back, and cervical regions.
The challenging diagnosis of cutaneous T-cell lymphoma, particularly mycosis fungoides (MF), stems from nonspecific clinical-laboratory findings11. Amorim GM, Quintella DC, Niemeyer-Corbellini JP, Ferreira LC, Ramos-e-Silva M, Cuzzi T. Validation of an algorithm based on clinical, histopathological and immunohistochemical data for the diagnosis of early-stage mycosis fungoides. An Bras Dermatol. 2020;95(3):326-31.. The annual incidence of T-cell cutaneous lymphoma is extremely low, at 0.77/100,000 individuals, with an estimated incidence of 0.41/100,000 for MF22. Lobato BADL, Brito JAGSM, Carneiro TX, Xavier MB. Diagnóstico tardio de micose fungoide: um relato de caso. Rev Pan-Amaz Saude. 2021;12:e202100820..
In this case, the variable symptomatic manifestations did not indicate early MF. Extensive desquamative plaques and ulcerated/infected lesions were the reasons for admission, while the detection of Sézary allowed formulation of a diagnostic hypothesis.
MF significantly resembles various benign inflammatory skin conditions33. Miyashiro D, Sanches JA. Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management. Front Oncol. 2023;13:1141108.. However, delayed diagnosis, as seen here, amplifies the likelihood of unfavorable outcomes44. Eklund Y, Aronsson A, Schmidtchen A, Relander T. Mycosis Fungoides: A Retrospective Study of 44 Swedish Cases. Acta Derm Venereol. 2016;96(5):669-73. Available from: https://doi.org/10.2340/00015555-2337
https://doi.org/10.2340/00015555-2337...
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Thus, a cautious approach is warranted in infectious diseases and dermatology settings, considering MF as a differential diagnosis in presentations hinting at it.
ETHICS
The study was approved by the Institutional Ethics Committee (CAAE 33818720.6.0000.5011).
ACKNOWLEDGMENTS
Not applicable.
REFERENCES
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1Amorim GM, Quintella DC, Niemeyer-Corbellini JP, Ferreira LC, Ramos-e-Silva M, Cuzzi T. Validation of an algorithm based on clinical, histopathological and immunohistochemical data for the diagnosis of early-stage mycosis fungoides. An Bras Dermatol. 2020;95(3):326-31.
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2Lobato BADL, Brito JAGSM, Carneiro TX, Xavier MB. Diagnóstico tardio de micose fungoide: um relato de caso. Rev Pan-Amaz Saude. 2021;12:e202100820.
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3Miyashiro D, Sanches JA. Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management. Front Oncol. 2023;13:1141108.
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4Eklund Y, Aronsson A, Schmidtchen A, Relander T. Mycosis Fungoides: A Retrospective Study of 44 Swedish Cases. Acta Derm Venereol. 2016;96(5):669-73. Available from: https://doi.org/10.2340/00015555-2337
» https://doi.org/10.2340/00015555-2337
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Financial Support: Not applicable.
Publication Dates
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Publication in this collection
25 Mar 2024 -
Date of issue
2024
History
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Received
22 Dec 2023 -
Accepted
16 Feb 2024