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Paraneoplastic Pemphigus

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Paraneoplastic Pemphigus, Neoplasia-Associated Pemphigus

  • See Also
  1. Bullous Disease
  2. Pemphigus
  • Epidemiology
  1. Incidence: Very rare
  2. Gender predominence: Female to male ratio of 2:1
  3. Age of onset: 60 years
  • 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
  • Signs
  1. Diffuse, painful bullous lesions
  2. Severe mucosal erosions in oropharynx and Conjunctiva
  3. Polymorphic target-like skin lesions
  • Differential Diagnosis
  1. Erythema Multiforme
  • Lab
  1. Histology
    1. Suprabasilar Acantholysis
    2. Dyskeratotic cells
    3. Basal cell vacuolization
  2. Immunofluorescence
    1. IgG and C3 on Keratinocyte cell surface
  • Prognosis
  1. Poor prognosis often with rapidly fatal course
  • References
  1. Parker in Goldman (2000) Cecil Medicine, p. 2285
  2. Bickle (2002) Am Fam Physician 65(9):1861-70 [PubMed]
  3. Cotell (2000) Am J Emerg Med 18(3): 288-99 [PubMed]