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Mycosis Fungoides / Sezary Syndrome

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Topic updated on 11/12/17 9:22am

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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