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Updated: Nov 12 2017

Mycosis Fungoides / Sezary Syndrome

  • Snapshot
    • A 55-year-old man complains of a rash he has had for many months. It occasionally itches. While he has tried some topical over-the-counter steroids, he has not seen his dermatologist yet since the symptoms were mild. On physical exam, his legs are covered with multiple well-demarcated red papules and plaques with some telangiectasias. A skin biopsy is obtained. A week later, the biopsy comes back with Pautrier microabsecesses, lymphocytic infiltrate, and cells with cerebriform nuclei.
  • Introduction
    • Both are variants of cutaneous T-cell lymphoma (CTCL)
      • helper CD4+ T-cell lymphoma of the skin
    • Mycosis fungoides (50-70% of CTCLs)
      • skin involvement only
    • Sézary syndrome (1-3% of CTCLs)
      • leukemic form of CTCL
      • CTCL + malignant T-cells in the blood
    • Epidemiology
      • older patients (median age 55-60)
      • more common in male patients
      • more common in black patients
  • Presentation
    • Symptoms
      • generalized pruritus
    • Physical exam
      • mycosis fungoides
        • well-demarcated, erythematous pruritic patches and plaques
        • in non-sun-exposed areas
        • can have telangiectasias and ulceration
        • erythroderma (> 90% body involvement)
        • early lesions may be confused with atopic dermatitis or psoriasis
      • Sézary syndrome
        • severely pruritic erythroderma with scaling
        • alopecia
        • lymphadenopathy
        • hepatosplenomegaly
  • Evaluation
    • Physical exam to assess for lymphadenopathy and organomegaly
    • Diagnosis usually made by clinical exam and history
    • Diagnosis confirmed with skin biopsy
      • lymphocytic infiltrate with Pautrier microabscesses within thickened epidermis.
      • buttock cells”: atypical lymphocytes with cerebriform nuclei (Sézary cells)
    • Labs
      • complete blood count with peripheral blood smear to look for Sézary cells (atypical T-cells)
  • Differential Diagnosis
    • Adult T-cell leukemia-lymphoma (ATLL)
    • Atopic dermatitis
    • Psoriasis
    • Drug eruption (SJS)
  • Treatment
    • Pharmacologic options
      • topical steroids
      • topical chemotherapies
      • radiation therapy
      • interferon
  • Prognosis, Prevention, and Complications
    • Prognosis
      • stage-dependent
      • mycosis fungoides typically indolent
        • slow-growing
      • Sézary syndrome typically aggressive
        • can be fatal
    • Complications
      • intense pruritus
      • secondary cutaneous bacterial or viral infection
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