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Fulminant purpuric rash
G Famularo1, C Pozzessere, V Trinchieri
1Department of Emergency Medicine, San Camillo Hospital, Circonvallazione Gianicolense, Rome, Italy.
Abstract:
We report on a fatal case of purpura fulminans caused by severe meningococcaemia. Despite early and aggressive treatment with the use of a specific algorithm and the maintenance of a stable haemodynamic status in the first hour since admission, purpura fulminans developed impressively over a few minutes. Necropsy showed microvascular thrombosis in the dermis but not in visceral organs, suggesting the diagnosis of meningococcal septic shock with purpura fulminans limited to the skin. Acquired deficiency of protein C, which exerts anticoagulant and antiinflammatory functions, is the central mechanism ultimately responsible for purpura fulminans. The disorder predicts a poor outcome of meningococcaemia and early and aggressive resuscitation is recommended in the emergency department with antibiotics, volume expansion, inotropic drugs, and protein C replacement. An attitude of scepticism is appropriate in the management of these patients even when early resuscitation is successful and haemodynamic parameters remain stable.
Insights
Purpura fulminans can rapidly develop in severe meningococcaemia despite early treatment. Acquired protein C deficiency is key, necessitating aggressive resuscitation and cautious management for meningococcal septic shock.
Area of Science:
- Infectious Diseases
- Hematology
- Critical Care Medicine
Background:
- Meningococcaemia, a severe bacterial infection, can lead to purpura fulminans.
- Purpura fulminans is a rare but life-threatening complication characterized by disseminated intravascular coagulation and skin necrosis.
Observation:
- A fatal case of purpura fulminans due to meningococcaemia is presented.
- Despite prompt and aggressive treatment, including hemodynamic support, purpura fulminans progressed rapidly.
Findings:
- Necropsy revealed dermal microvascular thrombosis, consistent with meningococcal septic shock and skin-limited purpura fulminans.
- Acquired deficiency of protein C, crucial for anticoagulant and anti-inflammatory functions, was identified as the central mechanism.
Implications:
- Purpura fulminans in meningococcaemia indicates a poor prognosis.
- Early, aggressive resuscitation in the emergency department is recommended, including antibiotics, volume expansion, inotropes, and protein C replacement.
- A skeptical approach to patient management is advised, even with initial resuscitation success.