What is Purpura Fulminans?
Purpura fulminans is an extreme type of rash characterized by its purplish discoloration. It’s said to be caused by the clotting of small blood vessels, leadings to such discoloration and to the skin decaying. It comes as a genuine skin emergency that requires immediate medical attention. Usually, patients with this condition are quite sick, and they may experience fever, bleeding from different areas, and low blood pressure. It often gets worse quickly and is regularly accompanied by blood clotting all over the body and shock from the heart not pumping enough blood.
This condition can affect newborns, children, and adults, and it has three different types based on what triggers its occurrence. Thanks to improvements in supportive care, the management of any other complications, and some specific treatments, the death rate from this disease has been decreasing. However, it’s still a debilitating condition and, unfortunately, it often leads to the need for major amputations in patients who survive, to prevent further complications.
What Causes Purpura Fulminans?
Purpura fulminans is a fast-progressing condition leading to blood clots and tissue death in the skin’s tiny blood vessels. However, it is not considered a disease on its own, but a symptom of certain serious illnesses. There are three types of this condition: neonatal, idiopathic, and acute infectious.
Neonatal purpura fulminans happens in newborns due to inherited deficiencies of certain proteins (protein C, protein S, and antithrombin III) that help prevent blood clotting. It shows up very early in life, and treatments focus on addressing these deficiencies.
The idiopathic type, we think, is a disorder that comes after an infection and is linked to the immune system attacking the body itself. This disorder typically follows an illness with fever and leads to rapidly appearing purpura (purple-colored spots or patches on the skin due to bleeding underneath). In this type, a deficiency in protein S, which is a protein that helps keep the blood from clotting excessively, is believed to be the cause.
The third type, acute infectious purpura fulminans, is the most common. It appears as a skin symptom in the most severe cases of sepsis, a life-threatening infection that can spread throughout the body, as well as necrotizing fasciitis, a severe skin infection that destroys tissue. This type tends to be related to certain infectious agents.
Risk Factors and Frequency for Purpura Fulminans
Purpura fulminans is a fairly uncommon disease that was first identified in 1884. Most of what we know about it comes from case reports and case series because it is not frequently seen. There are different kinds of purpura fulminans, each with its own rate of occurrence.
- The hereditary neonatal form, that severely lacks protein C, appears approximately once in every 1,000,000 live births.
- Acute infectious purpura fulminans may affect 10% to 20% of patients who have meningococcal septicemia, an extreme bacterial infection.
- In cases of acute infectious purpura fulminans, the most common bacterial causes are Meningococcus and Streptococcus pneumoniae, while the most frequent viral cause is varicella.
- Acute infectious purpura fulminans is more likely to occur in patients who lack a fully functioning spleen.
- The post-infectious form that occurs without a known cause is very rare, with only a few hundred cases reported.
Signs and Symptoms of Purpura Fulminans
Purpura fulminans is a skin condition characterized by specific signs and an evolving appearance. It starts with skin redness, which then forms irregular central zones of blue-black dead tissue due to severe bleeding. In some scenarios, blisters may appear. Initially, the affected skin feels hard and painful, but as the condition advances, sensation may be completely lost. Sometimes, dead tissue can get infected. The death of tissue may also affect deeper tissue layers.
When studying a case of purpura fulminans, doctors aim to recognize it early and find the root cause. They need to carefully examine the patient’s skin, keeping in mind other possible conditions like necrotizing fasciitis. In the early stages of related diseases, the skin signs might be hard to spot, so it’s crucial to maintain the possibility of purpura fulminans. Seeing a rash of tiny red spots or bruises in a baby or septic patient should prompt thoughts of purpura fulminans. A patient’s background can provide helpful information, such as a history of severe infections, injuries, or spleen removal surgeries.
- Because it can be linked with a condition called disseminated intravascular coagulation (DIC), patients may bleed from several places including mucous membranes, trauma sites, or the rectum
- Because it can be associated with severe septic shock, the patient may experience symptoms of damage to other body parts such as blood in the urine, reduced urine output, and difficulty breathing
- Severe pain not matching the physical findings should always make doctors consider necrotizing fasciitis
Testing for Purpura Fulminans
Purpura fulminans is a condition that affects the body’s ability to form blood clots. To confirm whether you have this, doctors usually test blood for levels of substances like antithrombin III, protein C and protein S. These levels can be especially helpful in diagnosing the disease in newborn babies.
In looking for what might have caused this condition, doctors often carry out tests like you would see with severe infections. They’ll send samples for lab testing and take pictures of the inside of your body. This can help them find out if there’s an infection causing it.
Sometimes, purpura fulminans can be similar to a severe skin infection called necrotizing fasciitis. In these cases, doctors might check the count of white blood cells, which help fight infection, and the level of sodium in your blood. They might also use pieces of information like these to decide if you need surgery right away.
Since purpura fulminans is often related to a condition where the body forms harmful blood clots throughout the body, doctors will also likely check for signs of this. They could check if there are too few platelets in the blood, test for higher than normal coagulation factors (these help your blood clot), see if there are an increased number of broken down blood clot pieces in your blood, and check if the level of a substance called fibrinogen, which helps form blood clots, is going down.
Treatment Options for Purpura Fulminans
When dealing with any type of purpura fulminans, a severe bleeding disorder, the first step involves supportive care and making sure the patient is properly hydrated. This is crucial due to the extensive blood clotting associated with this condition, which can affect multiple organs. It’s also vital to identify and address the root cause of the condition. To prevent further tissue death, blood thinners may be used. Depending on the situation, blood, blood clotting factors, and platelets might be needed due to the patient’s increased tendency to clot and a condition called disseminated intravascular coagulation (DIC), where blood clots form throughout the body. Quick surgical removal of dead tissues has been shown to decrease the chance of death.
For newborns with the disease, treatment typically involves hydrating the infant, giving them a platelet transfusion, and checking protein C and S levels – proteins in the blood which help prevent clotting. Fresh frozen plasma, which contains these proteins, may be required. Heparin and warfarin, types of blood thinners, are sometimes used. If tests show a lack of protein C, specific treatment to address this may be added.
For idiopathic purpura fulminans (when the cause is unknown), the treatment is quite similar. But, there might also be a benefit in using corticosteroids – medications that reduce inflammation and suppress the body’s immune system.
In the case of purpura fulminans caused by a severe infection, broad-spectrum antibiotics that can kill a wide range of bacteria may be used. These antibiotics are typically chosen to fight Neisseria meningitidis, Streptococcus, Staphylococcus, and Clostridia bacteria. Some possible antibiotics are carbapenem or a mixture of vancomycin with beta lactam-beta lactamase inhibitors. Clindamycin is often included because it can stop some of the toxins that cause this disease. IVIg therapy, which is a treatment made from donated blood that contains antibodies, is also used due to its ability to fight these toxins. Activated protein C might be given to lessen the inflammation, restore the balance of clotting in the body, and potentially slow down the skin injuries due to bleeding under the skin. In infectious purpura fulminans, the decision to use blood thinners depends on whether DIC is also present.
Throughout treatment for any type of purpura fulminans, the patient’s tissues are regularly checked, with dead areas removed as necessary. This often means multiple surgeries may be required.
What else can Purpura Fulminans be?
There are several conditions that could be related to the symptoms. These conditions include:
- Calciphylaxis
- Coumadin necrosis
- Meningococcemia
- Necrotizing fascitis
- Thrombotic thrombocytopenic purpura
- Toxic shock syndrome
- Thrombophlebitis
- Vasculitis
Remember, this is not an exhaustive list and specific symptoms can be related to different conditions.
