Erythema multiforme L51.0

Author: Prof. Dr. med. Peter Altmeyer

Our authors

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

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
This section has been translated automatically.

Ferdinand v. Hebra 1866

Definition
This section has been translated automatically.

Relatively frequent, polyetiological, mucocutaneous clinical picture characterized by an acute to sub-acute, self-limiting exanthema with characteristic, ring-in-ring structured spots and plaques and a possible mucosal infection. Strictly speaking, it is not an entity but a group of polyaetiological diseases with the described clinical leading symptoms, which is pathogenetically characterized by a common cytotoxic immune reaction directed against keratinocytes.

Occurrence/Epidemiology
This section has been translated automatically.

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

Etiopathogenesis
This section has been translated automatically.

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 biological methods HSV DNA could be detected in lesioned skin.

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

Associations with other infectious diseases such as HSV type II (HSV-2), varicella, parapoxvirus(Orf virus), parvovirus-B19, hepatitis C, histoplasma capsulatum, EBV infections, streptococci or mycoplasma (mycoplasma infections are more common in children) have been reported.

More rarely, severe mycotic or Gardnerella vaginalis infections are the cause.

It is not uncommon for vaccinations (mass-mumps-rubella vaccinations, hepatitis B vaccinations, vaccinations with the polyvalent HPV vaccine) to precede an EEM.

EEM is often observed in combination with infections after taking medication.

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

Manifestation
This section has been translated automatically.

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

Localization
This section has been translated automatically.

Back of hands, palms and soles of feet, neck, face and neck, extensor sides of the upper extremity; grouped in the area of the elbow, more rarely the knee. Mild (mostly oral) mucous membrane (lips, cheek mucous membrane and tongue) in about 50% of cases.

Clinical features
This section has been translated automatically.

Sudden onset without significant prodromies. Within 48-72 hours development of a disseminated, symptomless or slightly burning to itchy exanthema which may also occur in groups in the area of the elbow or knee. Initially 0.1-0.3 cm large reddish papules, which expand within 24 hours to form (almost pathognomic) shooting disc-shaped (cockades) plaques. Their color is reddish-livid, also hemorrhagic. In the centre of the plaques blistering is possible. Often a linear arrangement of the lesions is detectable ( Koebner phenomenon).

Histology
This section has been translated automatically.

The histological picture corresponds to that of classical acute cytotoxic interface dermatitis. A distinction must be made between:

  • Early stage: plexus-like orthokeratotic stratum corneum, oedema of the papillary body with a thin lymphocytic infiltrate, isolated eosinophil granulocytes, distinct exocytosis with compression of the lymphocytes in the lower epithelial layers. Few dyskeratotic keratinocytes (amorphous eosinophilic cytoplasm in pynotically condensed nuclei). Significant hydropic degeneration of the lower epithelial cell layers.
  • Later stage: plexus-like orthokeratotic stratum corneum, pronounced intra- and subepidermal edema up to subepithelial blistering; vacuum degeneration of the epidermis. Strong lymphocytic infiltrate with varying admixture of eosinophil granulocytes. Numerous dyskeratotic keratinocytes, which can also be aggregated to nests.

Differential diagnosis
This section has been translated automatically.

Therapy
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.

In most cases a local treatment with lotio alba is sufficient. In cases of severe itching and pronounced skin infestation, moderately strong glucocorticoid-containing topical preparations such as 0.1% triamcinolone cream (e.g. Triamgalen, Delphicort, R259 ) or 0.05-1% betamethasone emulsion (e.g. Betagalen, Betnesol, R030 ) are indicated. If the oral mucosa is affected, mouthwashes with anti-inflammatory preparations (e.g. chamomile extracts).

Internal therapy
This section has been translated automatically.

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

Progression/forecast
This section has been translated automatically.

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

Recurrent course is possible, with irregular occurrence of mostly 1-2 relapses/year, less frequently (up to 10 relapses/year).

More frequent episodes are observed in immunocompromised patients.

Some patients relapse 1 time per year in spring.

Note(s)
This section has been translated automatically.

Depending on the expressivity, severity and localisation of the skin and mucous membrane changes, different terms were used in the past, the originality of which is doubted today:

  • Erythema multiforme major
  • Dermatostomatitis Baader
  • Stevens-Johnson-Fuchs syndrome (syndrome muco-cutaneo-ocular fox)
  • Fiessinger-Rendu syndrome (Ectodermosis érosive pluriorificielle).

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

Literature
This section has been translated automatically.

  1. Ayangco L et al (2003) Oral manifestations of erythema multiforme. Dermatol Clin 21: 195-205
  2. 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,
  7. and treatment. Dent Clinic North Am 57:583-96.
  8. Seishima M, Oyama Z, Yamamura M (2001) Erythema multiforme associated with cytomegalovirus infection in nonimmunosuppressed patients. Dermatology 203: 299-302
  9. 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 of Devel Ther 8:2539-1547
  10. 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
  11. Trayes KP et al (2019) Erythema Multiforme: Recognition and Management. On Fam Physician. 100: 82-88.
  12. by Hebra F, Kaposi M (1860) Textbook of skin diseases. Volume 1, Enke, Erlangen

Disclaimer

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