What is Ecthyma Gangrenosum?
Ecthyma gangrenosum (EG) is a skin infection often found in individuals with weakened immune systems, washing through their blood quickly. It was first discovered by Dr. Lewellys Barker, a Canadian pathologist, in 1897 and primarily caused by the bacteria Pseudomonas aeruginosa. However, other bacteria have been found to potentially cause it since.
The infection appears as dead or dying ulcers with red borders. It mainly happens in the armpit and area around the genitals, but it can also occur on arms, legs, trunk, and face. Its characteristic look is due to the bacteria invading the blood vessels under the skin, interrupting the blood supply and causing the skin to die.
Immediate recognition and initial treatment with antibiotics that target a broad range of bacteria, especially Pseudomonas aeruginosa, are essential. Once lab results reveal the exact bacteria responsible and its sensitivity to specific antibiotics, the treatment can be adjusted to focus on the particular type identified.
It’s important to know that EG often suggests a poor outcome, especially for individuals with weakened immune systems and a low number of a specific type of white blood cells called neutrophils.
What Causes Ecthyma Gangrenosum?
Ecthyma gangrenosum, a skin condition, is most commonly caused by a bacterium called P. aeruginosa. Notably, one study discovered that this bacterium was found in 74% of diagnosed cases. P. aeruginosa is a specific kind of bacteria that doesn’t ferment lactose and is shaped like a rod. It particularly thrives in places where there’s moisture, like tap water, and it can be a problem in hospitals where it can spread via contaminated equipment, leading to infections.
However, P. aeruginosa is just one of several organisms that can cause ecthyma gangrenosum. Other bacteria that can cause this skin condition include MRSA (a type of antibiotic-resistant bacteria), Streptococcus pyogenes, Citrobacter freundii, Escherichia coli (commonly known as E. coli), Aeromonas hydrophila, Klebsiella pneumoniae, Serratia marcescens, Xanthomonas maltophilia, Morganella morganii, Corynebacterium diphtheriae, Neisseria gonorrhea, and Yersinia pestis.
It’s not just bacteria, though. Fungi, especially those from the Candida species, and viruses like the herpes simplex virus also have been known to cause ecthyma gangrenosum.
Risk Factors and Frequency for Ecthyma Gangrenosum
Ecthyma gangrenosum is a condition that can affect anyone, regardless of age or gender. However, people with weakened immune systems are especially at risk, with a large percentage of those with the condition having some form of immune deficiency. Even people with strong immune systems can get this disease. Certain conditions like low white blood cell count, leukemia, multiple myeloma, diabetes, malnutrition, and extensive burn wounds can increase the risk.
- Ecthyma gangrenosum can affect anyone, regardless of age or gender.
- People with weakened immune systems are most susceptible.
- Among those with the condition, 62% to 75% have an underlying immune deficiency.
- The disease can also occur in people with healthy immune systems.
- Conditions that increase the risk include low white blood cell count, leukemia, multiple myeloma, diabetes, malnutrition, and extensive burn wounds.
Signs and Symptoms of Ecthyma Gangrenosum
When diagnosing a condition, the doctor will take into account the timing and location of any skin lesions (abnormal growth or appearance), the patient’s immune system state, and any other symptoms such as coughing, fever, or pain during urination. If the condition is bacteremic (where bacteria is present in the bloodstream), it’s crucial to figure out where the infection came from. For example, a cough with mucus or painful urination might point to a lung or urinary infection source. In non-bacteremic (the bacteria is not present in the bloodstream) conditions, any recent injuries that might have allowed the bacteria to enter the body directly should be noted.
Ecthyma gangrenosum (EG) is a skin condition characterized by specific types of lesions. These start off as harmless-looking, round red spots or patches. However, these spots soon transform into pus-filled sores surrounded by redness. A bloody blister forms in the middle of the sore and then turns into a dead, ulcerated area with a black crust, or eschar. This changeover can sometimes happen very quickly, within 12 hours. The skin abnormalities can appear alone or in numerous places.
EG can develop anywhere on the body but usually is seen in the areas around the anus and genitals and the armpits. More than half of the cases (57%) present in the buttock or perineal area (the area between the anus and genitals), followed by the limbs (30%), the trunk of the body (6%), and the face (6%). Rare but reported cases have seen the area around the eyes affected. Doctors must examine the entire body for sores, paying special attention to fluctuant (fluid-filled) or extensively damaged areas.
Testing for Ecthyma Gangrenosum
If your doctor suspects you have Ecthyma gangrenosum (EG), a serious skin infection commonly linked to a weakened immune system, they will perform a number of tests. First, you may get blood and wound cultures, essentially samples of your blood and tissue, for testing. A complete overview of your blood cells and their functionality (a complete blood count) will also be performed along with a comprehensive metabolic panel, which is a blood test that measures your sugar level, electrolyte and fluid balance, and how your kidneys and liver are functioning.
In addition, a test for C-reactive protein will be conducted. C-reactive protein is produced by your liver and increases when there’s inflammation in your body. If you have this infection, this protein may be high. Your doctor may also test for HIV, since EG is more common in people with a weakened immune system.
If your doctor suspects sepsis, a life-threatening condition caused by the body’s response to an infection, they may also test your procalcitonin and lactate levels. Both these tests help evaluate the severity of an infection and are particularly important in cases of EG with sepsis.
Your doctor may also use a Wood’s lamp to inspect the lesions. This is a device that uses ultraviolet light to highlight bacteria, fungi, and other skin irregularities. A specific type of bacteria, Pseudomonas aeruginosa, that is often associated with EG, will show up as green under this light.
If any of these tests are unclear, or your symptoms are not typical, your doctor might recommend an excisional or punch biopsy. In these tests, small samples of tissue are removed from your body and analyzed under a microscope.
Treatment Options for Ecthyma Gangrenosum
If you’re suspected of having a skin condition called ecthyma gangrenosum, your doctor will swiftly start you on a wide-ranging antibiotic treatment after taking blood and wound sample to identify the exact cause of the infection.
At first, your treatment will broadly cover several different types of bacteria to make sure the actual cause is targeted. After the specific bacteria causing the infection is determined, your doctor will narrow down the list of antibiotics to more specific ones that can kill the particular bacteria causing the ecthyma gangrenosum.
If the infection is caused by a fungus or virus, which is quite rare, then specific treatments against these types of pathogens will be introduced.
In addition to antibiotics, some people might also need surgical treatment. If there are areas of dead skin (necrotic lesions) or pus-filled pockets (abscesses), your doctor might need to remove them. This is usually done through an incision or drainage method for abscesses, and the removal of all dead skin (debridement) and skin grafting (transplant of healthy skin) for necrotic skin lesions. Special care is given if lesions are larger than 10 cm.
Those with more severe cases, like those experiencing infection throughout their body (sepsis) or having low levels of a type of white blood cell called neutrophils (neutropenia), would likely be treated in an intensive care unit at a hospital due to the high risk of life-threatening consequences. However, if the ecthyma gangrenosum is not accompanied by an infection in the bloodstream, you might receive treatment in an outpatient setting. In this case, you may be given oral antibiotics like ciprofloxacin that can be taken at home.
What else can Ecthyma Gangrenosum be?
A detailed patient history, a physical examination, and basic lab tests are often sufficient to distinguish Ecthyma gangrenosum (EG) from other conditions that cause skin to die or breakdown. If a patient has low levels of neutrophils (a type of white blood cell) and cancer, and they also display typical skin symptoms, they may have EG. However, other conditions that can look very similar include:
- Autoimmune vasculitides (inflammation of the blood vessels)
- Vasopressor-induced necrosis (skin breakdown caused by medications used to raise blood pressure)
- Calciphylaxis (calcium buildup in the small blood vessels)
- Warfarin-induced necrosis (skin breakdown caused by a blood-thinning drug)
- Disseminated intravascular coagulation (a condition that leads to blood clots throughout the body)
- Pyoderma gangrenosum (a rare, painful skin disease)
- Necrolytic migratory erythema (a red, blistering rash that spreads across the skin)
- Livedoid vasculopathy (a rare disorder that causes painful sores on the legs)
In people with cancer and a low blood count, levels of fibrinogen (a blood clotting protein) and fibrin split products (d-dimer, a small protein fragment present in blood after a clot is degraded) could help distinguish between disseminated intravascular coagulation and EG. In individuals in intensive care receiving vasopressors, the location of the lesions can also be helpful; vasopressor-induced necrosis affects the ends of the limbs, while EG usually affects the areas around the genitals, anus, and underarms. In some cases, a tissue sample may be necessary to confirm the diagnosis if it isn’t clear from less invasive methods.
What to expect with Ecthyma Gangrenosum
Developing Esophageal Gangrene (EG) lesions when already experiencing sepsis (a severe infection) generally leads to a poor prognosis. In fact, in such situations, the condition can prove to be fatal for 20% to 77% of patients. However, in situations where someone with EG doesn’t also have a bacterial infection (non-bacteremic EG), the mortality rate is significantly lower; only 8%.
The outcome is highly dependent on the patient’s immune system. For example, when patients are already dealing with health problems that weaken their immune systems (like cancer, kidney failure, and so forth), they are often more severely affected.
The most crucial factor influencing the mortality rate is the presence of neutropenia at the time of diagnosis. Neutropenia is a condition characterized by unusually low levels of a type of white blood cell called neutrophils, which are essential for fighting off infections.
Possible Complications When Diagnosed with Ecthyma Gangrenosum
EG is a very serious infection that can potentially lead to a condition where your blood pressure drops drastically, known as septic shock. It’s crucial for doctors to quickly identify and start treating this condition with the right antibiotics for the best possible result.
- Very serious infection
- Potential for drastic blood pressure drop
- Quick recognition and treatment by healthcare professionals is essential
- Appropriate use of antibiotics for treatment
Preventing Ecthyma Gangrenosum
It’s important for patients to know that if their immune system is weakened – which is what being ‘immunocompromised’ means – they need to take the best possible care of their underlying health condition. Generally, these patients are cared for in a hospital environment to ensure they receive the right treatment and management for their condition.