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.

See below Erythema multiforme

Erythema multiforme majus is usually a single episode and often an expression of a cytotoxic drug-induced hypersensitivity reaction (e.g. 5-fluorouracil and actinomycin D).

EM-majus has also been observed in combination 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. Here too, CD8+ driven, granzyme B-dependent, drug-specific cytotoxic activity against keratinocytes plays a significant role. It leads to keratinogen apoptosis. This epidermal cell death in EM/SJS/TEN lesions occurs primarily via the Fas-Fas ligand (FasL) and perforin/granzyme signaling pathways. The blister fluid also contains a large amount of granulysin. Other proaptotic factors are the cytokines TNF-alpha, interferon-gamma and annexin A1. The annexin A1 released by monocytes interacts with the formyl peptide receptor 1.

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

Extremities (often emphasized on the extensor side), in children also on the trunk; 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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