Vegetative pyoderma gangrenosum
Synonyms: Granulomatous 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 a solitary, erythematous, ulcerated plaque, which lacks the violaceous border typically present in classic pyoderma gangrenosum, usually affecting individuals who are otherwise healthy. Histologically, the lesion presents a central layer containing neutrophilic inflamation, surrounded by a palisade of histiocytes, which are rimmed by a lymphocytic infiltrate. In comparison with the other variants of pyoderma gangrenosum, this subtype usually shows a good response to less aggressive treatments and underlying systemic disorders are less frequently associated. It is considered the most benign and uncommon clinical variant of pyoderma gangrenosum.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vegetative pyoderma gangrenosum | Granulomatous pyoderma gangrenosum |
| fr | Pyoderma gangrenosum, type végétatif | Pyodermite granulomateuse superficielle |
| de | Pyoderma gangraenosum, vegetatives | Pyoderma gangraenosum, granulomatöses |
| es | Pioderma gangrenoso vegetante | Pioderma gangrenoso granulomatoso |
| it | Pioderma gangrenoso vegetativo | |
| nl | Vegetatieve pyoderma gangrenosum | Granulomateuze pyoderma gangrenosum |
| pt | Pioderma gangrenoso vegetativo | |
| pl | Piodermia wegetatywna zgorzelinowa | = en |
| cs | Vegetativní pyoderma gangrenosum | Granulomatózní pyoderma gangrenosum |
| uk | Вегетативна гангренозна піодермія | Гранульоматозна гангренозна піодермія |
| zh | 植物性坏疽性脓皮病 | 肉芽肿性坏疽性脓皮病 |
| tr | Vegetatif piyoderma gangrenozum | Granülomatöz 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 | 0035238 | E | validated | |
| UMLS | C5680158 | 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 / 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/538872
- This record as JSON: /data/d/538872.json