Erythema multiforme (overview) L51.0

Ferdinand v. Hebra first described erythema exsudativum multiforme in his textbook in 1860 as a purely cutaneous, polymorphic erythema (exanthema) with a benign course. In 1876, clinically more severe, similar cases were described with skin and mucous membrane involvement (erythema multiforme majus). In 1922, the syndrome described by Stevens and Johnson, a febrile exanthema with stomatitis and purulent conjunctivitis, was published. In 1956, the term "toxic epidermal necrolysis" was created by Lyell (Lyell syndrome). Recently, there has been a tendency to combine Stevens-Johnson syndrome and toxic epidermal necrolysis under the generic term EN - epidermal necrolysis.

Erythema multiforme is a polyetiologic, mucocutaneous inflammatory syndrome triggered by an infection-induced hyperergic immune reaction. Clinically, EM is characterized by an acute to subacute, self-limited exanthema prone to recurrence with characteristic, well-defined, targetoid (disc-in-disc structure with heterogeneous ring formations, possibly also central blistering) and localized blistering. (disc-in-disc structure with heterogeneous ring formations, possibly also central blistering) as well as localized but also confluent plaques, maximum 1.0-3.0 cm in size (so-called typical cockades), and possible mucosal involvement (oral mucosa, conjunctiva, genital and anal mucosa). The classic erythema multiforme(EM minor variant) is usually mild and is mainly caused by herpes simplex viruses but also other viruses and bacteria (e.g. mycoplasma). Involvement of the lips/mouth mucosa can be detected in 20-70% of patients. A few patients suffer a recurrent form with recurring attacks over months and years. Erythema multiforme majus is much more severe with skin and varying degrees of mucosal involvement (conjunctiva, labial and oral mucosa, anal and genital mucosa).

Erythema (exsudativum) multiforme minor: mild (classic) form, which is mainly triggered by herpes simplex infections. Involvement of the lip and oral mucosa is observed in 20-70% of cases. Some cases recur. A recurrent course over many years is possible (recurrent erythema multiforme)

Erythema (exsudativum) multiforme majus: this severe, usually single, extremity variant with severe erosive mucosal involvement is also predominantly virus-induced. Typical and/or atypical cockades are detectable. In children and adolescents, Mycoplasma pneumoniae may be the cause (EM strain)

Fuchs syndrome: In this clinical variant of erythema multiforme major (first described in 1906), there is either localized involvement of the conjunctiva (and oral mucosa). By definition, skin symptoms recede into the background. Note: The diagnosis is less common today. Instead, this sympotmatism is classified as a minus variant of EM-majus.

Ectodermosis erosiva pluriorificialis (Rendu-Fiessinger): Clinical variant of erythema multiforme major with polytopic involvement of the oral-genital and anal mucosa (term no longer in use today).

Reactive infectious mucocutaneous eruption (RIME): Clinical variant of erythema multiforme major which is mainly caused by Mycoplasmae pneumoniae.

Mycoplasma-induced rash and mucositis (MIRM ), an inflammatory mucocutaneous eruption in children and adolescents associated with infections caused by Mycoplasma pneumoniae. Other associated pathogens that cause this inflammatory symptomatology have since been identified. The name RIME is preferred.

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