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Essentials of Diagnosis
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General Considerations
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A cutaneous T cell lymphoma that begins on the skin and may involve only the skin for years or decades (so-called mycosis fungoides)
Certain medications (including selective serotonin reuptake inhibitors) and photoallergy may produce eruptions clinically and histologically identical to those of mycosis fungoides, so this possibility must always be considered
Lymph node enlargement may be due to benign expansion of the node (dermatopathic lymphadenopathy) or by specific involvement with mycosis fungoides
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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 or nondiagnostic 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
Lymphadenopathy may occur locally or widely
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Differential Diagnosis
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Therapeutic 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:
PUVA plus retinoids
PUVA plus interferon
Extracorporeal photophoresis
Bexarotene
Histone deacetylase inhibitors (romidepsin or vorinostat)
Targeted immunomodulators (brentuximab, mogamulizumab)
Total skin electron beam
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Usually slowly progressive (over decades)
Early and aggressive treatment has not been proved to cure or prevent progression of the disease
Prognosis is better in patients with patch or plaque stage disease and worse in patients with erythroderma, tumors, and lymphadenopathy
Survival is not reduced in patients with limited patch disease
Elderly patients with limited patch and plaque stage disease commonly die of other causes
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