Erythema multiforme L51.0

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 02.11.2023

Dieser Artikel auf Deutsch


Baader's dermatitis; Cockade erythema; Dermatostomatitis Baader; Disc rose; EEM; Erythema exsudativum multiforme; Erythema multiforme from Herbra; Erythema multiforme minor; Erythema multiforme postherpetic; Fuchs Syndrome; hidroa vesiculosa; Postherpetic erythema exsudativum multiforme

This section has been translated automatically.

Ferdinand v. Hebra 1866

This section has been translated automatically.

Relatively common, polyetiologic, mucocutaneous reaction pattern characterized by an acute to subacute, self-limited exanthema prone to recurrence with characteristic, slice-like structured (slice-in-slice structure) patches, plaques and blisters as well as possible mucosal involvement. Strictly speaking, it is not an entity but a group of polyetiologic diseases with the described leading clinical symptoms, which are pathogenetically characterized by a common cytotoxic immune reaction directed against keratinocytes with epidermal "saddle cell necrosis".

This section has been translated automatically.

Mainly occurring in young adults, rarely in children. m>w; no ethnic prevalence known.

This section has been translated automatically.

Genetics: There is evidence of a genetic predisposition of EEM with the following HLA associations: HLA-DQw3, DRw53, AW33.

Infections: In the majority of adolescents and adults, herpes simplex type 1 (HSV-1) infections precede exanthema or become clinically apparent after the onset of exanthema. By molecular biology methods, HSV DNA has been detected in lesional skin. Associations with other infectious diseases such as HSV type II (HSV-2), varicella, parapoxviruses(Orf virus), parvovirus-B19, hepatitis C, SARS-CoV-2 (Thielmann CM et al. 2022), Histoplasma capsulatum, EBV infections, streptococci, or mycoplasma (mycoplasma infections occur frequently in children) have been reported. Less commonly, severe mycotic or Gardnerella vaginalis infections are causative.

Vaccinations: Not very rarely, vaccinations (masen-mumps-rubella vaccinations, hepatitis B vaccinations, vaccinations with the polyvalent HPV vaccine) precede erythema multiforme.

Medications: EM is frequently observed after medication (e.g. 5-fluorouracil and actinomycin D ) gfls. also in combination with viral or bacterial infections (Wang S et al. (2022) .

Multiforme "scatter reactions" may also occur in allergic contact dermatitis (e.g., paraphenylenediamine in tattoos).

This section has been translated automatically.

Occurs mainly in young adults, low androtrophy. Frequency peak: 20 - 40 LJ. Rarely in infants.

This section has been translated automatically.

Back of the hands, palms and soles, neck, face and neck, extensor sides of the upper extremity; grouped in the area of the elbow, rarely the knee. The trunk may also be affected (see Fig.). Mild (usually oral) mucosal involvement (lips, buccal mucosa and tongue) in about 50% of cases.

Clinical features
This section has been translated automatically.

Sudden onset without significant prodromes. Within 48-72 hours, formation of a disseminated, usually strictly symmetrical exanthema, which may occur in groups in the area of the elbow or knee and is asymptomatic or slightly burning to itchy.

Initial 0.1-0.3 cm reddish papules that expand within 24 hours to form (almost pathognomic) disc-shaped (cocard) plaques.

Their color is reddish-livid, also hemorrhagic. Blistering is possible in the center of the plaques. A linear arrangement of the lesions is often detectable ( Köbner phenomenon).

This section has been translated automatically.

The histologic picture corresponds to that of classic acute cytotoxic interface dermatitis.

To be distinguished are:

  • Early stage: Basket-weave orthokeratotic stratum corneum, edema of the papillary body with sparse lymphocytic infiltrate, scattered eosinophilic granulocytes, marked exocytosis with condensation of lymphocytes in the lower epithelial layers. Few dyskeratotic keratinocytes (amorphous eosinophilic cytoplasm with pyknotic condensed nuclei). Marked hydropic degeneration of the lower epithelial cell layers.
  • Later stage: Basket-weave orthokeratotic stratum corneum, marked intra- and subepidermal edema to subepithelial blistering; vacuolar degeneration of epidermis. Vigorous lymphocytic infiltrate with variable admixture of eosinophilic granulocytes. Numerous dyskeratotic keratinocytes, which may also be aggregated into nests.

Differential diagnosis
This section has been translated automatically.

This section has been translated automatically.

It is an acute, self-liminating disease. In this respect, symptomatic therapy is generally sufficient.

External therapy
This section has been translated automatically.

Local treatment with Lotio alba is usually sufficient. For more severe itching and pronounced skin infestation, medium-strength glucocorticoid-containing externals such as 0.1% triamcinolone cream (e.g., Triamgalen, Delphicort,Triamcinolone acetonide cream hydrophilic 0.025/0.05/0.1% (NRF 11.38.), or 0.05-1% betamethasone emulsion (e.g., Betagalen®, Betnesol, betamethasone valerate emulsion hydrophilic 0.025/0.05 or 0.1% (NRF 11.47.) are indicated. If the oral mucosa is affected, mouth rinses with antiphlogistic preparations (e.g., chamomile extracts).

Internal therapy
This section has been translated automatically.

If symptoms are pronounced, systemic glucocorticoids such as prednisone (e.g. Decortin Tbl.) 50-75 mg/day. Additionally, in case of itching, antihistamines, e.g. desloratadine (Aerius), levocetirizine (Xyzall), cetirizine (Zyrtec) p.o. In case of frequent (postherpetic) recurrences, oral prophylaxis with aciclovir for 1 year is indicated (10 mg/kg bw/day) (evidence level: IB).

This section has been translated automatically.

Favorable. Self-limiting course with complete healing after 10 to 14 days.

Recurrent course is possible, with irregular occurrence of usually 1-2 recurrences/year, rarely more frequent (up to 10 recurrences/year).

More frequent episodes are observed in immunosuppressed individuals.

Some patients relapse 1 time/year in spring.

This section has been translated automatically.

Depending on the expressivity, severity and localization of the skin and mucosal changes, different terms were used in the past, the independence of which is now doubted:

  • Erythema multiforme major
  • Baader's dermatostomatitis
  • Stevens-Johnson-Fuchs syndrome (Syndroma muco-cutaneo-oculare Fuchs)
  • Fiessinger-Rendu syndrome (ectodermosis érosive pluriorificielle).

These variants, together with Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN), are now considered a spectrum of disease with varying degrees of severity.

This section has been translated automatically.

  1. Ayangco L et al (2003) Oral manifestations of erythema multiforme. Dermatol Clin 21: 195-205
  2. Das S et al (2000) Herpes simplex virus type 1 as a cause of widespread intracorneal blistering of the lower limbs. Clin Exp Dermatol 25: 119-121
  3. Hidajat C et al (2014) Drug-mediated rash: erythema multiforme versus Stevens-Johnson syndrome. BMJ Case Rep 22 PubMed PMID: 25246464.
  4. Johnston GA et al (2002) Neonatal erythema multiforme major. Clin Exp Dermatol 27: 661-664.
  5. Molnar I, Matulis M. (2002) Arthritis associated with recurrent erythema multiforme responding to oral acyclovir. Clin Rheumatol 21: 415-417.
  6. Samim F et al.(2013) Erythema multiforme: a review of epidemiology, pathogenesis, clinical features, and treatment. Dent Clin North Am 57:583-96.
  7. Seishima M, Oyama Z, Yamamura M (2001) Erythema multiforme associated with cytomegalovirus infection in nonimmunosuppressed patients. Dermatology 203: 299-302
  8. Sun J et al (2014) Stevens-Johnson syndrome and toxic epidermal necrolysis: a multi-aspect comparative 7-year study from the People's Republic of China. Drug Des Devel Ther 8:2539-1547.
  9. Tatnall FM et al (1995) A double-blind, placebo-controlled trial of continous acyclovir therapy in recurrent erythema multiforme. Br J Dermatol 132: 267-270
  10. Thielmann CM et al (2022) COVID-19-triggered EEM-like skin lesions. Dtsch Arztebl Int 119:131.

  11. Trayes KP et al (2019) Erythema multiforme: recognition and management. Am Fam Physician. 100: 82-88.
  12. Von Hebra F, Kaposi M (1860) Textbook of skin diseases. Vol 1, Enke, Erlangen
  13. Wang S et al. (2022) 5-Fluorouracil and actinomycin D lead to erythema multiforme drug eruption in chemotherapy of invasive mole: Case report and literature review. Medicine (Baltimore) 101:e31678


Please ask your physician for a reliable diagnosis. This website is only meant as a reference.


Last updated on: 02.11.2023