Paraneoplastic Pemphigus

Aka: Paraneoplastic Pemphigus, Neoplasia-Associated Pemphigus

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II. Epidemiology

  1. Incidence: Very rare
  2. Gender predominence: Female to male ratio of 2:1
  3. Age of onset: 60 years

III. 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

IV. Signs

  1. Diffuse, painful bullous lesions
  2. Severe mucosal erosions in oropharynx and Conjunctiva
  3. Polymorphic target-like skin lesions

V. Differential Diagnosis

  1. Erythema Multiforme

VI. Lab

  1. Histology
    1. Suprabasilar Acantholysis
    2. Dyskeratotic cells
    3. Basal cell vacuolization
  2. Immunofluorescence
    1. IgG and C3 on Keratinocyte cell surface

VII. Prognosis

  1. Poor prognosis often with rapidly fatal course

VIII. 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]

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