Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Erythema multiforme major
Synonyms

Erythema multiforme majus

Erythema exsudativum multiforme majus

Definitions

A rare skin disease characterized most typically by targetoid papules with 3-rings of concentric color variation (dark center, surrounded by lighter pink ring and an outer red ring) symmetrically distributed on the extensor surfaces of the extremities, or more diffuse, accompanied by 2 or more mucosal involvements (erosions of the oral, genital, anal mucosa, conjunctivitis, pseudomembranes). The condition is commonly proceeded by prodromal symptoms of malaise, fever, and myalgias, and is usually self-limiting, resolving within a few weeks; although, there is a risk of recurrence. The disease is mostly triggered by infectious agents (herpes virus, <i>mycoplasma pneumoniae</i>) but a subset of patients have auto-antibodies targeting plakins. Rare cases of severe mucosal sequelae have been described.

ID

http://www.orpha.net/ORDO/Orphanet_502499

alternative_term

Erythema multiforme majus

Erythema exsudativum multiforme majus

definition

A rare skin disease characterized most typically by targetoid papules with 3-rings of concentric color variation (dark center, surrounded by lighter pink ring and an outer red ring) symmetrically distributed on the extensor surfaces of the extremities, or more diffuse, accompanied by 2 or more mucosal involvements (erosions of the oral, genital, anal mucosa, conjunctivitis, pseudomembranes). The condition is commonly proceeded by prodromal symptoms of malaise, fever, and myalgias, and is usually self-limiting, resolving within a few weeks; although, there is a risk of recurrence. The disease is mostly triggered by infectious agents (herpes virus, mycoplasma pneumoniae) but a subset of patients have auto-antibodies targeting plakins. Rare cases of severe mucosal sequelae have been described.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=502499

hasDbXref

ICD-10:L51.8

UMLS:C3241919

label

Erythema multiforme major

notation

ORPHA:502499

part_of

http://www.orpha.net/ORDO/Orphanet_293815

prefixIRI

ORDO:Orphanet_502499

prefLabel

Erythema multiforme major

treeView

http://www.orpha.net/ORDO/Orphanet_293815

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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