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Essentials of Diagnosis
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Localized or generalized erythematous patches or scaly plaques
Sometimes associated with pruritus, lymphadenopathy
Distinctive histology
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General Considerations
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A cutaneous T-cell lymphoma that begins on the skin and may remain there for years or decades (so-called mycosis fungoides)
It may progress to systemic disease, including Sézary syndrome (erythroderma with circulating malignant T cells)
Early and aggressive treatment has not been proven to cure or prevent disease progression
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Localized or generalized erythematous patches or plaques, usually on the trunk
Plaques are almost always over 5 cm in diameter
Pruritus is a frequent complaint and can be severe
The lesions often begin as nondescript patches, and it is not unusual for the patient to have skin lesions for more than a decade before the diagnosis can be confirmed
Follicular involvement with hair loss is characteristic of mycosis fungoides
In more advanced cases, tumors appear
Local or diffuse lymphadenopathy may be due to
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Differential Diagnosis
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Diagnostic Procedures
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The treatment is complex
Skin-directed therapies are used initially, including
Topical corticosteroids
Topical mechlorethamine
Bexarotene gel
UV phototherapy
If the disease progresses, the following may be used:
Psoralen and UVA (PUVA) plus retinoids
PUVA plus interferon
Extracorporeal photophoresis
Bexarotene
Histone deacetylase inhibitors (romidepsin or vorinostat)
Targeted immunomodulators (brentuximab, mogamulizumab)
Total skin electron beam therapy
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Usually slowly progressive (over decades)
Prognosis is better with patch or plaque stage disease and worse with erythroderma, tumors, and lymphadenopathy
Survival is not reduced in patients with limited patch disease
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Kempf
W
et al. Cutaneous T-cell lymphomas—an update 2021. Hematol Oncol. 2021;39:46.
[PubMed: 34105822]
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Valipour
A
et al. Interventions for mycosis fungoides. Cochrane Database Syst Rev. 2020;7:CD008946. ...