Erythema multiforme majus L51.1

Acute, usually severe (up to max. 10% of KOF) mucocutaneous inflammatory syndrome characterized by a self-limited exanthema with characteristic, well-defined, target-like (disc-in-disc structure with heterogeneous ring formations, possibly also central blistering) and pronounced mucosal involvement (oral mucosa, conjunctiva, genital and anal mucosa). A transition to toxic epidermal necrolysis is excluded.

In the mycoplasma-induced rash and mucositis ( MIRM) variant, a predominance of enanthema, blistering, erosion and crusting can be observed, with the oral mucosa (94%), the eye region (82%) and the urogenital area (63%) being particularly affected. Infestation of the nasal introitus and anus is less common. Mucosal lesions are generally characterized as ulcerative or haemorrhagic and can cause symptoms. Involvement of the nose can manifest itself in the form of firm hemorrhagic crusts. Anal lesions can lead to pain during bowel movements.

See below Erythema multiforme

In the case of erythema multiforme majus, which is characterized by extremity, the herpes simplex virus is a possible cause. In children, adolescents and young adults, Mycoplasma pneumoniae must be considered as a trigger with a high percentage. EM-majus can also be observed in combination of drugs with viral or bacterial infections (Wang S et al. 2022). In these cases, overlaps with other clinical pictures such as Stevens-Johnson syndrome or toxic epidermal necrolysis should be noted.

As with SJS, CD8+ driven, granzyme B-dependent, drug-specific cytotoxic activity against keratinocytes plays a significant role. It leads to keratinogenic apoptosis. Multiforme "scattering reactions" can also occur in allergic contact dermatitis (e.g. paraphenylenediamine in tattoos).

Extremities (often accentuated on the extensor side), in children and adolescents (Mycoplasma pneumomiae as trigger) also trunk accentuation; involvement of the mucous membranes close to the surface (conjunctiva, labial and oral mucous membranes, anal and genital mucous membranes).

See below Erythema multiforme

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