Erythema multiforme major
Synonyms: Erythema exsudativum multiforme majus · Erythema multiforme majus
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Erythema multiforme major | Erythema exsudativum multiforme majus · Erythema multiforme majus |
| fr | Erythème polymorphe majeur | |
| de | Erythema multiforme major | = en |
| es | Eritema multiforme mayor | Eritema exudativo multiforme mayor · Eritema multiforme majus |
| it | Eritema multiforme maggiore | |
| nl | Erythema multiforme major | = en |
| pt | Eritema multiforme major | |
| pl | Rumień wielopostaciowy większy | Rumień wielopostaciowy wysiękowy |
| cs | Erythema multiforme major | Erythema multiforme majus · Erythema exsudativum multiforme majus |
| uk | Мультиформна ексудативна еритема | Ексудативна мультиформна велика еритема · Мультиформна велика еритема |
| zh | 多发性红斑 | 渗出性多发性大面积红斑 |
| tr | Eritema multiforme majör | Eritema multiforme majus · Eritema eksudativum multiforme majus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L51.8 | NTBT | attributed | validated |
| ICD-11 | EB12.1 | E | specific | validated |
| MONDO | 0044719 | E | validated | |
| UMLS | C3241919 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:293815Toxic dermatosis
rare skin diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/502499
- This record as JSON: /data/d/502499.json