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KEY FEATURES

Essentials of Diagnosis

  • Localized or generalized erythematous patches or scaly plaques

  • Sometimes associated with pruritus, lymphadenopathy

  • Distinctive histology

General Considerations

  • 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

CLINICAL FINDINGS

Symptoms and Signs

  • 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

    • Benign expansion (dermatopathic lymphadenopathy)

    • Involvement with mycosis fungoides

Differential Diagnosis

  • Psoriasis

  • Drug eruption

  • Photoallergy

  • Atopic dermatitis (eczema)

  • Syphilis

  • Tinea corporis (body ringworm)

DIAGNOSIS

Laboratory Tests

  • Circulating malignant T cells (Sézary cells) can be detected in the blood (T cell gene rearrangement test) in advanced disease

  • Eosinophilia may be present

Diagnostic Procedures

  • Diagnosis is based on skin biopsy though numerous biopsies may be required before the diagnosis can be confirmed

TREATMENT

Medications

  • 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

OUTCOME

Complications

  • Overly aggressive treatment may lead to complications and premature demise

Prognosis

  • 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

When to Refer

  • All patients with suspected or proven diagnosis

REFERENCES

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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. ...

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