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

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

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Ontology: 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, シュヨウズイハンセイテンポウソウ
Czech Paraneoplastický pemfigus
Hungarian Paraneoplasiás pemphigus