Herpetiform pemphigus
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare superficial pemphigus disease characterized by severe intractable pruritus with erythematous or urticarial plaques and sometimes vesicles organized in a herpetiform pattern. Mucosae are generally spared. Eosinophilia in peripheral blood and low titers of circulating autoantibodies are observed in many cases. Histology can show an aspect of either pemphigus (superficial or deep), or an intraepidermal infiltrate rich in eosinophils (eosinophilic spongiosis).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Herpetiform pemphigus | |
| fr | Pemphigus herpétiforme | |
| de | Pemphigus herpetiformis | |
| es | Pénfigo herpetiforme | |
| it | Pemfigo erpetiforme | |
| nl | Pemphigus herpetiformis | Herpetiforme pemfigus |
| pt | Pênfigo herpetiforme | |
| pl | Pęcherzyca opryszczkowata | |
| cs | Pemphigus herpetiformis | |
| uk | Герпетиформний пемфігус | |
| zh | 疱疹样天疱疮 | |
| tr | Herpetiform pemfigus |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L10.8 | NTBT | attributed | validated |
| ICD-11 | EB40.Y | NTBT | attributed | validated |
| MONDO | 0016164 | E | validated | |
| UMLS | C4749730 | 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:46485Superficial pemphigus
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/208524
- This record as JSON: /data/d/208524.json