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Start free with EleplanAcquired purpura fulminans
ORPHA:49566Disease
What it is
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.
Key facts
- Age of onset
- Adult, Childhood, Infancy, Neonatal
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Common30–79%
19- Abnormal blistering of the skin
- Abnormal thrombosis
- Acrocyanosis
- Decreased erythrocyte sedimentation rate
- Disseminated intravascular coagulation
- Elevated circulating C-reactive protein concentration
- Erythematous macule
- Gangrene
- Hypofibrinogenemia
- Immune dysregulation
- Macular purpura
- Macule
- Prolonged partial thromboplastin time
- Prolonged prothrombin time
- Reduced protein C activity
- Reduced protein S activity
- Shock
- Skin rash
- Thrombocytopenia
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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