Erythema multiforme L51.0

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 12.05.2024

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Synonym(s)

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

History
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Ferdinand v. Hebra 1866

Definition
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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".

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

Etiopathogenesis
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Genetics: There is evidence of a genetic predisposition to 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 the exanthema or appear clinically after the onset of the exanthema. HSV DNA has been detected in lesional skin using molecular biological methods. 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 more frequently in children) have been reported. Severe mycotic or Gardnerella vaginalis infections are less common. In a cross-sectional analysis (n= 43,547 patients with a history of COVID-19 infection), the risk of EM was 6.68 times higher than in patients without COVID-19 (Saleh W et al. 2024).

Vaccinations: Vaccinations (mumps-rubella vaccinations, hepatitis B vaccinations, vaccinations with the polyvalent HPV vaccine) often precede erythema multiforme. At 2.7, the risk of developing EM after a COVID-19 vaccination is significantly higher than in the general population. The prevalence of EM after a COVID-19 infection or vaccination differs significantly from that of the general population (Saleh W et al. 2024).medication: EM is often observed after taking medication (e.g. 5-fluorouracil and actinomycin D ), sometimes in combination with viral or bacterial infections (Wang S et al. (2022) .

Multiforme "scattering reactions" can also occur with allergic contact eczema (e.g. paraphenylenediamine in tattoos).

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

Localization
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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
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Sudden onset without significant prodromes. Within 48-72 hours, a disseminated, usually symmetrical, asymptomatic or slightly burning to itchy exanthema develops, which may also occur in groups in the elbow or knee area. The exanthema develops rapidly and relapses within a few days with a few to hundreds of lesions. A linear arrangement of the lesions is possible (Köbner phenomenon).

Initially there are reddish papules 0.1-0.3 cm in size, which expand to plaques several centimeters in size within 24 hours. Their color is reddish-livid, also hemorrhagic. The center can sink in, show a tendency to regress, but can also show the opposite development, intensify with a purple component or blistering. Concentric rings (shooting target phenomenon - association with cockades) develop as a result of bursts of activity emanating from the center. The EM lesions do not form necroses and heal without scarring within a few days to weeks.

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

External therapy
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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
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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).

Progression/forecast
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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.

Note(s)
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Depending on the expressivity, severity and localization of the skin and mucous membrane changes, different terms were used in the past, the independence of which is doubted today:

  • Erythema multiforme major
  • Dermatostomatitis Baader
  • Stevens-Johnson-Fuchs syndrome (syndroma muco-cutaneo-oculare Fuchs)
  • Fiessinger-Rendu syndrome (ectodermosis érosive pluriorificielle).

Together with Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN), these variants are now regarded as a separate disease spectrum with varying degrees of severity.

Literature
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  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. Labé P et al (2020) Erythema multiforme and Kawasaki disease associated with COVID-19 infection in children. J Eur Acad Dermatol Venereol 34:e539-e541
  6. Lavery MJ et al. (2021) A flare of pre-existing erythema multiforme following BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine. Clin Exp Dermatol 46:1325-7.
  7. Molnar I, Matulis M. (2002) Arthritis associated with recurrent erythema multiforme responding to oral acyclovir. Clin Rheumatol 21: 415-417
  8. Saleh W et al. (2024) Increased prevalence of erythema multiforme in patients with COVID-19 infection or vaccination. Sci Rep 14:2801.
  9. Samim F et al.(2013) Erythema multiforme: a review of epidemiology, pathogenesis, clinical features, and treatment. Dent Clin North Am 57:583-96.
  10. Seishima M, Oyama Z, Yamamura M (2001) Erythema multiforme associated with cytomegalovirus infection in nonimmunosuppressed patients. Dermatology 203: 299-302
  11. 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
  12. Tatnall FM et al. (1995) A double-blind, placebo-controlled trial of continuous acyclovir therapy in recurrent erythema multiforme. Br J Dermatol 132: 267-270
  13. Thielmann CM et al (2022) COVID-19-Triggered EEM-Like Skin Lesions. Dtsch Arztebl Int 119:131.
  14. Trayes KP et al. (2019) Erythema Multiforme: Recognition and Management. Am Fam Physician. 100: 82-88.
  15. von Hebra F, Kaposi M (1860) Textbook of skin diseases. Volume 1, Enke, Erlangen
  16. 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

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