Mycosis Fungoides

Aka: Mycosis Fungoides, Cutaneous T-Cell Lymphoma, Sezary Syndrome, Sezary Cell, Sezary Erythroderma

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II. Pathophysiology

  1. T-Cell Lymphoma that begins in skin
  2. Sezary Syndrome is leukemic form of T-Cell Lymphoma

III. Epidemiology

  1. Incidence: 0.42 cases per 100,000 (rare)
  2. Most common after age 50 years
  3. Gender: Twice as common in men
  4. Black patients are more commonly affected

IV. Symptoms

  1. Pruritus

V. Signs (four stages of development)

  1. Eczematous patches and Plaques
  2. Distributed over non-sun-exposed skin
    1. Buttocks
    2. Thighs
  3. Tumors develop later in course
    1. Associated with superficial and deep Lymphadenopathy
    2. Associated with metastases to Spleen, lung, GI Tract

VI. Complications

  1. Exfoliative Dermatitis

VII. Course

  1. Survival <3 years after tumors develop

VIII. References

  1. Habif (1996) Clinical Dermatology, Mosby, p. 674-5
  2. Pujol (2000) J Am Acad Dermatol 42:324-8 [PubMed]

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