Dermatology Book

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Paraneoplastic Pemphigus

Aka: Paraneoplastic Pemphigus, Neoplasia-Associated Pemphigus
  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):1861-70 [PubMed]
    3. Cotell (2000) Am J Emerg Med 18(3): 288-99 [PubMed]

Paraneoplastic pemphigus (C1112570)

Concepts Neoplastic Process (T191)
ICD10 L10.81
LNC LP94506-0
English Paraneoplastic pemphigus, paraneoplastic pemphigus, paraneoplastic pemphigus (diagnosis), pemphigus paraneoplastic
Dutch paraneoplastische pemphigus
French Pemphigus paranéoplasique
German paraneoplastischer Pemphigus
Italian Pemfigo paraneoplastico
Portuguese Pênfigo paraneoplásico
Spanish Pénfigo paraneoplásico
Japanese 腫瘍随伴性天疱瘡, ｼｭﾖｳｽﾞｲﾊﾝｾｲﾃﾝﾎﾟｳｿｳ
Czech Paraneoplastický pemfigus
Hungarian Paraneoplasiás pemphigus
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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