Summary about Disease
Purpura Fulminans (PF) is a rare, life-threatening condition characterized by rapidly progressive skin necrosis, disseminated intravascular coagulation (DIC), and shock. It's a medical emergency requiring immediate treatment. PF often occurs as a complication of severe infections (especially bacterial meningitis in children) or can be associated with inherited deficiencies in coagulation proteins. It leads to widespread blood clotting in small vessels, causing tissue damage and organ dysfunction.
Symptoms
Rapidly developing purpura: Characterized by large, purple-colored spots and bruises on the skin that spread quickly.
Pain: Severe pain in the affected areas.
Skin necrosis: The skin turns black and dies.
Fever or hypothermia: Body temperature can be very high or very low.
Shock: Rapid heart rate, low blood pressure, and altered mental status.
Bleeding: Bleeding from the nose, gums, or injection sites.
Organ dysfunction: Kidney failure, respiratory distress, and other organ problems may occur.
Causes
Purpura Fulminans can be caused by several factors:
Infections:
Bacterial (e.g., Meningococcemia, Sepsis)
Viral (e.g., Chickenpox, Measles)
Rickettsial
Inherited deficiencies:
Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Drug-induced:
Warfarin-induced skin necrosis (rare)
Idiopathic:
In some cases, the cause is unknown.
Medicine Used
Treatment for Purpura Fulminans is complex and may include:
Antibiotics: If the cause is bacterial infection.
Anticoagulants: Heparin is often used to manage DIC.
Protein C concentrate: If the patient has Protein C deficiency.
Fresh Frozen Plasma (FFP): To replace clotting factors.
Blood transfusions: To correct anemia and thrombocytopenia.
Vasopressors: To maintain blood pressure.
Surgical debridement: Removal of dead tissue.
Skin grafting: May be needed to repair damaged skin.
Is Communicable
Purpura Fulminans itself is not communicable. However, if it is caused by an infection (such as meningococcal disease), that underlying infection can be communicable. In such cases, close contacts of the patient may need prophylactic antibiotics.
Precautions
Precautions depend on the underlying cause:
If caused by an infection: Standard infection control measures are essential to prevent the spread of the infection (e.g., hand hygiene, respiratory precautions). Prophylactic antibiotics for close contacts may be necessary.
For patients with inherited deficiencies: Genetic counseling and monitoring may be required.
General precautions: Avoiding trauma to prevent further skin damage. Careful monitoring for signs of infection or bleeding.
How long does an outbreak last?
The "outbreak" refers to the acute episode of Purpura Fulminans itself. The active phase of PF, involving rapid progression of skin lesions, DIC, and shock, typically lasts for days to weeks. The duration is heavily dependent on the speed of diagnosis, the promptness and effectiveness of treatment, and the underlying cause. The recovery phase, involving wound healing and rehabilitation, can take much longer.
How is it diagnosed?
Diagnosis is based on clinical findings and laboratory tests:
Clinical examination: Observing the characteristic skin lesions.
Blood tests:
Complete blood count (CBC) to assess platelet count and anemia.
Coagulation studies (PT, PTT, fibrinogen, D-dimer) to evaluate DIC.
Blood cultures to identify bacterial infections.
Protein C, Protein S, and antithrombin levels to check for inherited deficiencies.
Skin biopsy: To confirm the diagnosis and rule out other conditions.
Lumbar puncture: To rule out meningitis if infection is suspected.
Imaging studies: May be done to assess for internal organ involvement.
Timeline of Symptoms
The progression of symptoms can be rapid:
Initial phase: Fever, malaise, and sometimes symptoms of the underlying infection.
Early stage: Development of small, red spots (petechiae) that quickly progress to larger, purplish bruises (purpura).
Rapid progression: Purpura expands rapidly, often becoming confluent. Pain and skin necrosis develop.
Severe stage: DIC, shock, and organ dysfunction occur. This timeline can occur over hours to a few days.
Important Considerations
Emergency: Purpura Fulminans is a medical emergency and requires immediate medical attention.
Early diagnosis and treatment are crucial: Delay in diagnosis and treatment can lead to severe complications and death.
Underlying cause: Identifying and treating the underlying cause is essential.
Multidisciplinary approach: Management requires a multidisciplinary team including infectious disease specialists, hematologists, surgeons, and intensivists.
Long-term complications: Survivors may experience long-term complications such as skin scarring, limb amputations, and neurological sequelae.