Cutaneous small vessel vasculitis
Synonyms: Cutaneous hypersensitivity vasculitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A small vessel vasculitis characterized by neutrophilic inflammation predominantly limited to the superficial cutaneous postcapillary venules and without systemic vasculitis or glomerulonephritis. Typical presentation is of unifocal or multifocal palpable purpura on the lower extremities.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Cutaneous small vessel vasculitis | Cutaneous hypersensitivity vasculitis |
| fr | Vascularite des petits vaisseaux cutanés | Vascularite hypersensitive cutanée |
| de | Kutane Vaskulitis kleiner Gefäße | Vaskulitis, kutane hypersensitive |
| es | Vasculitis cutánea de pequeño vaso | Angeítis cutánea de pequeño vaso · Angeítis cutánea por hipersensibilidad · Vasculitis cutánea por hipersensibilidad |
| it | Vasculite cutanea dei piccoli vasi | Angioite ipersensibile · Vasculite cutanea dei piccoli vasi · Vasculite ipersensitiva cutanea · Vasculite leucocitoclastica cutanea |
| nl | Cutane vasculitis van kleine bloedvaten | Cutane overgevoeligheidsvasculitis |
| pt | Vasculite dos pequenos vasos cutâneos | Vasculite cutânea |
| pl | Leukocytoklastyczne zapalenie naczyń skórnych | Skórne leukocystoklastyczne zapalenie naczyń · Zapalenie małych naczyń skórnych · Zapalenie naczyń skórnych z nadwrażliwością · Zapalenie naczyń z nadwrażliwością |
| cs | Kožní leukocytoklastická angiitida | Kožní hypersenzitivní vaskulitida · Kožní leukocytoklastická vaskulitida · Kožní vaskulitida malých cév · Hypersenzitivní angiitida |
| uk | Шкірний васкуліт дрібних судин | Шкірний васкуліт гіперчутливості |
| zh | 皮肤小血管炎 | 皮肤过敏性血管炎 |
| tr | Kutanöz küçük damar vasküliti | Kutanöz aşırı duyarlılık vasküliti |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M31.0 | NTBT | attributed | validated |
| ICD-11 | EF40.1 | E | specific | validated |
| MONDO | 0019509 | E | validated | |
| MONDO | 19509 | E | validated | |
| UMLS | C0151436 | E | validated | |
| GARD | 7851 | 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:156149Immune complex mediated vasculitis
rare systemic and rheumatological diseases; rare circulatory system 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 |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 2.7 / 100 000 | Norway | Value and class | Validated | 9805179[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | 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
14 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000965 | Cutis marmorata | |
| HP:0010783 | Erythema | |
| HP:0001945 | Fever | |
| HP:0100758 | Gangrene | |
| HP:0003326 | Myalgia | |
| HP:0200034 | Papule | |
| HP:0000979 | Purpura | |
| HP:0001581 | Recurrent skin infections | |
| HP:0001025 | Urticaria | |
| HP:0002633 | Vasculitis | |
| Frequent (79-30%) | ||
| HP:0002829 | Arthralgia | |
| HP:0000988 | Skin rash | |
| Occasional (29-5%) | ||
| HP:0000163 | Abnormal oral cavity morphology | |
| HP:0001482 | Subcutaneous nodule | |
Run the differential on the 10 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/889
- This record as JSON: /data/d/889.json