Acquired purpura fulminans
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood, Infancy, Neonatal
- Status
- active
A life-threatening, rapidly progressive thrombotic disorder affecting mainly neonates and children that is characterized by purpuric skin lesions and disseminated intravascular coagulation. It may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. There are two forms of the disorder that are classified according to triggering mechanisms: acute infectious (the most common form), and idiopathic purpura fulminans.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acquired purpura fulminans | |
| fr | Purpura fulminans acquis | |
| de | Purpura fulminans, erworbene | |
| es | Púrpura fulminante adquirido | |
| it | Porpora fulminante acquisita | |
| nl | Verworven purpura fulminans | |
| pt | Púrpura fulminante | |
| pl | plamica piorunująca nabyta | |
| cs | Získaná purpura fulminans | |
| uk | Набута фульминантна пурпура | |
| zh | 获得性暴发性紫癜 | |
| tr | Edinsel purpura fulminans |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D65 | NTBT | attributed | validated |
| ICD-11 | 3B20 | NTBT | attributed | validated |
| MONDO | 0018854 | E | validated | |
| UMLS | C4510896 | E | validated | |
| MedDRA | 10037556 | 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:248365Rare thrombotic disorder due to an acquired coagulation factors defect
rare hematological 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 |
|---|---|---|---|---|---|---|
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Point prevalence | Unknown | — | Worldwide | Class only | 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
25 annotations · validated 2019-05-22| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0008066 | Abnormal blistering of the skin | |
| HP:0001977 | Abnormal thrombosis | |
| HP:0001063 | Acrocyanosis | |
| HP:0025022 | Decreased erythrocyte sedimentation rate | |
| HP:0005521 | Disseminated intravascular coagulation | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0025475 | Erythematous macule | |
| HP:0100758 | Gangrene | |
| HP:0011900 | Hypofibrinogenemia | |
| HP:0002958 | Immune dysregulation | |
| HP:0031365 | Macular purpura | |
| HP:0012733 | Macule | |
| HP:0003645 | Prolonged partial thromboplastin time | |
| HP:0008151 | Prolonged prothrombin time | |
| HP:0005543 | Reduced protein C activity | |
| HP:0004855 | Reduced protein S activity | |
| HP:0031273 | Shock | |
| HP:0000988 | Skin rash | |
| HP:0001873 | Thrombocytopenia | |
| Occasional (29-5%) | ||
| HP:0001399 | Hepatic failure | |
| HP:0011029 | Internal hemorrhage | |
| HP:0002170 | Intracranial hemorrhage | |
| HP:0002664 | Neoplasm | |
| HP:0100806 | Sepsis | |
| Very rare (<4-1%) | ||
| HP:0025452 | Pyoderma gangrenosum | |
Annotation source: 2142890[PMID]_17679737[PMID]_20034317[PMID]_23582572[PMID]_29157918[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/49566
- This record as JSON: /data/d/49566.json