PsAPASH syndrome
Synonyms: Psoriatic arthritis-pyoderma gangrenosum-acne-hidradenitis suppurativa syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare autoinflammatory syndrome characterized by the combination of psoriatic arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis (which, in addition to axillae and inguinal folds, can be observed in other areas, such as the buttocks or labia majora).
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | PsAPASH syndrome | Psoriatic arthritis-pyoderma gangrenosum-acne-hidradenitis suppurativa syndrome |
| fr | Syndrome PsAPASH | Syndrome d'arthrite psoriasique-pyoderma gangrenosum-acné-hidradénite suppurée |
| de | PsAPASH-Syndrom | Psoriasis-Arthritis-Pyoderma gangrenosum-Akne-Hidradenitis suppurativa-Syndrom |
| es | Síndrome PsAPASH | Síndrome de artritis psoriásica-pioderma gangrenoso-acné-hidradenitis supurativa |
| it | Sindrome PsAPASH | Sindrome artrite psoriasica- pioderma gangrenoso-acne-idrosadenite suppurativa |
| nl | PsAPASH-syndroom | Artritis psoriatica - pyoderma gangrenosum - acne - hidradenitis suppurativa-syndroom · Psoriasisartritis - pyoderma gangrenosum - acne - hidradenitis suppurativa-syndroom |
| pt | Síndrome PsAPASH | Síndrome de artrite psoriática-pioderma gangrenoso-acne-hidradenite supurativa |
| pl | Zespół PsAPASH | = en |
| cs | PsAPASH syndrom | = en |
| uk | Синдром PsAPASH | Псоріатичний артрит - гангренозна піодермія - акне - гнійничковий синдром - гідраденіт |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L88 | NTBT | attributed | validated |
| ICD-11 | EB21 | NTBT | attributed | validated |
| MONDO | 0958257 | E | validated | |
| MONDO | 958257 | E | validated | |
| UMLS | C5816786 | 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:653434Autoinflammatory syndrome with acne and/or hidradenitis suppurativa
rare skin diseases; rare systemic and rheumatological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 10 cases | Worldwide | Case(s) | Validated | 32942279[PMID]_ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 32942279[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/641390
- This record as JSON: /data/d/641390.json