Classic pyoderma gangrenosum
Synonyms: Ulcerative pyoderma gangrenosum
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare subtype of pyoderma gangrenosum disease characterized by rapidly progressive, single or multiple, painful, aseptic ulcers which present overhanging, violaceous and undermined borders, surrounding induration and erythema, and granulation tissue (occasionally necrotic tissue and/or a purulent exudate) at the base, mainly affecting the legs (but other body surfaces may also be involved), leading to chronic ulcerations and often regressing with cribriform mutilating scars. The disease presents a chronic relapsing course and systemic features (e.g. fever, malaise, arthralgia, myalgia) may be associated.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic pyoderma gangrenosum | Ulcerative pyoderma gangrenosum |
| fr | Pyoderma gangrenosum, type classique | Pyoderma gangrenosum, type ulcéreux |
| de | Pyoderma gangraenosum, klassisches | Pyoderma gangraenosum, ulzeratives |
| es | Pioderma gangrenoso clásico | Pioderma gangrenoso ulcerativo |
| it | Pioderma gangrenoso classico | |
| nl | Klassieke pyoderma gangrenosum | Ulceratieve pyoderma gangrenosum |
| pt | Pioderma gangrenoso clássico | |
| pl | Klasyczna piodermia zgorzelinowa | = en |
| cs | Klasická pyoderma gangrenosum | Ulcerózní pyoderma gangrenosum |
| uk | Класична гангренозна піодермія | Виразкова гангренозна піодермія |
| zh | 经典坏疽性脓皮病 | 溃疡性坏疽性脓皮病 |
| tr | Klasik piyoderma gangrenozum | Ülseratif piyoderma gangrenozum |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | L88 | NTBT | attributed | validated |
| ICD-11 | EB21 | NTBT | index | validated |
| MONDO | 0035235 | E | validated | |
| UMLS | C5680157 | 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:48104Pyoderma gangrenosum
rare genetic diseases; rare skin diseases; rare systemic and rheumatological diseases
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| PTPN6 | Disease-causing germline mutation(s) in | Assessed | HGNC:9658 | ENSG00000111679 | 176883 | 12p13.31 | protein tyrosine phosphatase non-receptor type 6 | 21406173[PMID] |
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET_25213386[PMID] |
| Annual incidence | 1-9 / 1 000 000 | — | Italy | Class only | Validated | ORPHANET_29877043[PMID] |
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/538863
- This record as JSON: /data/d/538863.json