Pemphigus vulgaris

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: EB40.0Z

  • Wichmann, 1793
  • Hebra, 1860

Rare, severe blistering autoimmune disease in which autoantibodies are directed against structural proteins of the skin and mucosa. Unlike pemphigoid, intraepithelial cleavage occurs.

  • All pemphigus diseases together
    • Incidence: 1-2/1,000,000 / year
    • Of these, 80% are pemphigus vulgaris patients, after which pemphigus foliaceus is the most common
  • Incidence
    • Switzerland: 0.6-0.76 / million inhabitants / year
    • Germany: 1.5/million population/year
    • Greece: 8.0/ million inhabitants / year
    • Individual religion-related population groups: 16.1- 32 / million inhabitants / year
    • M = F
  • Age peak: 40th-60th yrs

  • Autoantibodies against desmoglein 1 and/or 3 destroy desmoglein, which functions as an adhesion molecule for keratinocytes
  • Associations:
    • Autoimmune diseases
      • Myasthenia gravis
      • Thymoma
      • Lupus erythematosus
    • Neoplasms
      • Lymphomas
      • Carcinomas
    • Medicines, esp
      • D-penicillamine
      • Rifampicin
      • ACE inhibitors
      • Diclofenac
      • Propanolol
      • Indometacin
      • Pyrazolone derivatives
      • Cephalosporins
    • Physical stimuli
      • Burns
      • UV irradiation
      • X-ray irradiation
    • HLA class II alleles DQB1, DRB1

  • Localisation
    • Mucous membranes (oral cavity and genital), umbilicus, intertrigines. Potentially, the entire remaining integument may be affected
  • Both on the mucous membranes (involvement in 50%), but also cutaneously, blisters are often not visible initially. Rather, erosions, aphthae or "eczematous" skin changes appear. In the course, blistering, erosions and crust formation occur. Secondary infections can lead to scarring and healing
  • If there is laryngeal involvement, hoarseness may occur; if there is oesophageal involvement, there may be difficulty swallowing.
  • Nikolski's phenomenon I: Lateral spatula pressure causes bullae to be triggered: positive
  • Nikolski's phenomenon II: Bullae can be displaced by lateral spatula pressure: positive

  • Anamnesis according to S2k- AWMF guideline:
    • Time of the first appearance of the lesions
    • Pain (where, when)
    • Stomatitis, dysphagia, hoarseness?
    • Conjunctivitis?
    • Epistaxis?
    • Dysuria?
    • Weight loss?
    • Medication history?
    • Ethnic background
  • Clinical
  • Biopsy
    • Dermatopathology (small intact blister by biopsy)
    • Direct immunofluorescence (4 mm punch biopsy from perilesional skin or mucosa (<1cm adjacent to a blister)
  • Indirect immunofluorescence (IF)
    • Monkey oesophagus
    • Desmoglein 3-ELISA (no western blot recommended)
    • Desmoglein 1-ELISA (no Western blot recommended)

There are > 50 non-desmoglein target antigens which can/should be determined if appropriate.

Recommendations according to S2-guideline

Direct immunofluorescence Desmoglein 1 and 3 antibodies Determination of anti-desmocollin antibody
+ - Determination may be recommended (not mandatory)
- - No recommendation

Mucous membranes (oral cavity and genital), umbilicus, intertrigines. Potentially the entire remaining integument can be affected.

  • Suprabasal acantholysis. Inflammatory infiltrates and oedema are visible in the corium
  • Direct immunofluorescence
    • IgG deposits (and often C1, C3, C4, rarely IgM or IgA) in the intercellular space of the epidermis

  • Potentially lethal
  • Rarely complete healing

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