Erythema exsudativum multiforme

Last Updated: 2023-07-07

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

ICD11: EB12.Z

Ferdinand v. Hebra 1860

Erythema multiforme, EM, EEM, erythema multiforme of Hebra, cocard erythema, disk rose.

Acute, usually self-limiting, polyaetiological disease characterised by "target-like lesions".

  • Division:
    • Minor form: no mucosal involvement.
    • Major form: with mucosal involvement (up to 70% of patients).

  • Incidence: unknown, but estimated well below 1% per annum.
  • Peak incidence: 20- 40 yrs.
  • M > F

  • Possible triggers (combinations also possible)
  • Infections
    • approx. 90% of all triggers
    • Viral
      • Herpes simplex virus (HSV) type I (most common trigger)
      • HSV type II
      • Orf virus
      • Parapox virus
      • Varicella zoster virus
      • Epstein-Barr virus
      • Parvovirus B19
      • Adenovirus
      • Cytomegalovirus
      • Coxsackie virus
      • Hepatitis B or C- virus
      • HI virus
    • Bacteria
      • Mycoplasma pneumoniae (frequent trigger, especially in children)
      • Chlamydophila psittaci
      • Salmonella
    • Mycobacteria
      • Mycobacterium tuberculosis
    • Mycoses
      • Histoplasma capsulatum
      • Dermatophytes
    • Medicines (< 10%)
      • NSAIDs
      • Sulfonamides
      • Antiepileptics
      • Antibiotics
    • Neoplasia
    • Autoimmune diseases
    • Vaccinations
    • UV-radiation (sunburn)
    • Trauma
    • Sarcoidosis
    • Menstruation
    • Possible genetic predisposition: HLA-DQw3, DRw53, AW33, HLA-DQB1*0301, B15(62), B35, and DQ3.
    • HSV infection causes cellular immune responses against viral antigens localised in the skin.

  • Localisation
    • Mostly symmetrical on the extensor sides of the extremities. Spread is centripetal. Face, trunk, palmoplantar, elbows as well as mucous membranes may be affected.
  • After an incubation period of 2-3 days, the first skin changes usually appear. In some cases, however, there may be up to 17 days between contact with the trigger and the first appearance of efflorescences.
  • Facultative prodromal symptoms may occur:
  • ↓ AZ, fever, myalgias
  • Cough in M. pneumoniae infection
  • There are usually erythematous, often pruritic or burning papules in the above-mentioned localisations, which in the course of time change exanthematously into coquette- and disc-shaped plaques with a characteristic central vesicle. A linear arrangement may be detectable (Köbner phenomenon)
  • The area of the mucous membranes shows enanthema, painful erosions or vesicles. Usually only the oral cavity is affected, but in a few cases it can lead to involvement of the pharynx and the upper respiratory organs.
  • In most cases, only the oral mucosa is affected. Involvement of the genital mucosa occurs in approx. 25%, ocular involvement in approx. 17%.

  • Anamense (infections?, herpes simplex?, cough?, medication?)
  • Clinic
  • Lab:
    • Nonspecific
    • Optional: ↑BSG, leukocytosis, ↑ transaminases, HSV serology pos.
  • U status
    • Mycoplasma pneumoniae
  • Biopsy
    • Dermatopathology
    • Direct immunofluorescence

  • Scarless healing, but in some cases with hyperpigmentation, within 2 weeks.
  • Very few patients suffer recurrences, some up to 6x per year. The average duration of recurrences is 6-10 years.

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