PASS syndrome
Synonyms: Pyoderma gangrenosum-acne-hidradenitis suppurativa-ankylosing spondylitis syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare autoinflammatory syndrome characterized by a chronic-relapsing course of the combination of pyoderma gangrenosum, acne, hidradenitis suppurativa (which, in addition to axillae and inguinal folds, can be observed on upper and lower limbs, back, and buttocks, among others), and ankylosing spondylitis. Typical age of onset is adolescence to young adulthood, with the different signs and symptoms appearing simultaneously or subsequently.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | PASS syndrome | Pyoderma gangrenosum-acne-hidradenitis suppurativa-ankylosing spondylitis syndrome |
| fr | Syndrome PASS | Syndrome de pyoderma gangrenosum-acné-hidradénite suppurée-spondylarthrite ankylosante |
| de | PASS-Syndrom | Pyoderma gangrenosum-Akne-Hidradenitis suppurativa-ankylosierende Spondylarthritis-Syndrom |
| es | Síndrome PASS | Síndrome de pioderma gangrenoso-acné-hidradenitis supurativa-espondilitis anquilosante |
| it | Sindrome PASS | Sindrome pioderma gangrenoso-acne- idrosadenite suppurativa-spondilite anchilosante |
| nl | PASS-syndroom | Pyoderma gangrenosum - acne - hidradenitis suppurativa - ankyloserende spondylitis-syndroom |
| pt | Síndrome PASS | Síndrome de pioderma gangrenoso-acne-hidradenite supurativa-espondilite anquilosante |
| pl | Zespół PASS | = en |
| cs | PASS syndrom | = en |
| uk | Синдром PASS | Синдром гангренозної піодермії - акне - гнійного гідраденіту - анкілозуючого спондиліту |
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 | 0958256 | E | validated | |
| MONDO | 958256 | E | validated | |
| UMLS | C5816788 | 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 | 16 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/641385
- This record as JSON: /data/d/641385.json