Preferred Label : Purpura fulminans;
ICD-11 definition : A fulminant form of disseminated intravascular coagulation with widespread purpura
progressing rapidly to large confluent areas of cutaneous necrosis. It is typically
associated with meningococcal septicaemia but may occur in association a range of
other disorders including systemic automimmmune diseases and antiphospholipid autoantibodies.
NEW: Purpura fulminans is a life-threatening, rapidly progressive thrombotic disorder
affecting mainly neonates and children, characterized by purpuric skin lesions and
disseminated intravascular coagulation. The lesions are initially well-demarcated
erythematous macules, rapidly developing irregular central areas of blue-black hemorrhagic
necrosis, becoming painful, dark and raised, sometimes with vesicle or bulla formation.
Lesions later lead to irreversible endothelial ischemic injury with gangrenous necrosis.
Purpura fulminans may progress rapidly to multi-organ failure by thrombotic occlusion
of small and medium-sized blood vessels. There are three forms classified by triggering
mechanism: neonatal, acute infectious (the most common form), and idiopathic.;
Origin ID : 1293848111;
Currated CISMeF NLP mapping
- Validated automatic mappings to BTNT
- Validated automatic mappings to NTBT
A fulminant form of disseminated intravascular coagulation with widespread purpura
progressing rapidly to large confluent areas of cutaneous necrosis. It is typically
associated with meningococcal septicaemia but may occur in association a range of
other disorders including systemic automimmmune diseases and antiphospholipid autoantibodies.
NEW: Purpura fulminans is a life-threatening, rapidly progressive thrombotic disorder
affecting mainly neonates and children, characterized by purpuric skin lesions and
disseminated intravascular coagulation. The lesions are initially well-demarcated
erythematous macules, rapidly developing irregular central areas of blue-black hemorrhagic
necrosis, becoming painful, dark and raised, sometimes with vesicle or bulla formation.
Lesions later lead to irreversible endothelial ischemic injury with gangrenous necrosis.
Purpura fulminans may progress rapidly to multi-organ failure by thrombotic occlusion
of small and medium-sized blood vessels. There are three forms classified by triggering
mechanism: neonatal, acute infectious (the most common form), and idiopathic.
