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Paraneoplastic PemphigusAka: Neoplasia-Associated Pemphigus

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  1. See Also
    1. Bullous Disease
    2. Pemphigus
  2. Epidemiology
    1. Incidence: Very rare
    2. Gender predominence: Female to male ratio of 2:1
    3. Age of onset: 60 years
  3. Pathophysiology
    1. Autoantibodies to Desmoplakin 1 and 3
    2. Lymphoid malignancy associated Pemphigus
      1. Lymphoma
      2. Thymoma
      3. Chronic Lymphocytic Leukemia
      4. Waldenstrom's Macroglobulinemia
      5. Sarcoma
      6. Castleman's Disease
  4. Signs
    1. Diffuse, painful bullous lesions
    2. Severe mucosal Erosions in oropharynx and Conjunctiva
    3. Polymorphic target-like skin lesions
  5. Differential Diagnosis
    1. Erythema Multiforme
  6. Lab
    1. Histology
      1. Suprabasilar Acantholysis
      2. Dyskeratotic cells
      3. Basal cell vacuolization
    2. Immunofluorescence
      1. IgG and C3 on Keratinocyte cell surface
  7. Prognosis
    1. Poor prognosis often with rapidly fatal course
  8. References
    1. Parker in Goldman (2000) Cecil Medicine, p. 2285
    2. Bickle (2002) Am Fam Physician 65(9):1861
    3. Cotell (2000) Am J Emerg Med 18(3):288

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