Pemphigus is a topic covered in the 5-Minute Emergency Consult.

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Basics

Description

  • Autoantibody (IgG)-mediated blistering disease of the skin and mucous membrane:
    • Characterized by loss of cell-to-cell adhesion called acantholysis
  • Median age 71 yr
    • Reports of disease occurring in neonates through elderly
  • Rare; worldwide incidence 0.7/100,000
  • Females > males, 66 vs. 34%
  • Pemphix is Greek for bubble or blister
  • Pemphigus, specific term for autoantibody disease against some portion of epidermis
  • Pemphigoid: A term describing the group of syndromes that cause a separation of the epidermis from the dermis, typically more benign course
  • Mortality is highest in those with mucocutaneous involvement
    • If untreated, mortality rates average 60–90%, with treatment this nears 5%
  • 3 major subtypes exist:
    • Vulgaris; typically more serious with deeper mucocutaneous involvement:
      • Accounts for 70–80% of all pemphigus
      • Up to 70% with vulgaris present with oral lesions, which is often the presenting complaint
      • Autoantibodies to Dsg 1 and 3
      • Affects most races in middle age and elderly Ashkenazi Jews
    • Foliaceus; milder and more superficial cutaneous lesions:
      • Oral lesions and better prognosis
      • Autoantibodies to Dsg 1 only
    • Paraneoplastic pemphigus; often with severe mucocutaneous involvement
      • Most commonly seen in lymphoreticular malignancies

Pediatric Considerations
  • Pemphigus is rare in neonates and children but may occur in adolescents
  • Early diagnosis and treatment significantly impact growth, psychological, social, and cultural development
  • Histopathology is identical to adult disease
  • Neonates may develop the disease secondary to transplacental transfer of IgG
  • Neonatal pemphigus spontaneously resolves in several weeks as the maternal antibodies are catabolized


Pregnancy Considerations
Effective treatment of maternal disease prior to conception lowers the risk of neonatal transmission and gestational complications

Etiology

  • IgG autoantibodies are directed against desmosomal cadherins desmoglein 1 and desmoglein 3 found in all keratinocytes
  • Autoantibodies cause histopathologic acantholysis, cytoskeletal derangements, and apoptosis
  • Bullae formation is caused by the loss of cell–cell adhesion and separation of the keratinocytes
  • Immunogenetic predisposition secondary to higher frequencies of specific human leukocyte antigen HLA haplotypes including DR4 and DRw6
  • Drugs such as penicillamine, captopril, rifampin, piroxicam, and phenobarbital can trigger pemphigoid reactions
  • Endemic pemphigus foliaceus (fogo selvagem), most common in South America, may be triggered or transmitted by bites from flying insects
  • Pemphigoid reactions may occur in association with a neoplasm, usually lymphoma (paraneoplastic pemphigus)

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Citation

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TY - ELEC T1 - Pemphigus ID - 307347 Y1 - 2016 PB - 5-Minute Emergency Consult UR - https://emergency.unboundmedicine.com/emergency/view/5-Minute_Emergency_Consult/307347/all/Pemphigus ER -