What is Pyoderma Gangrenosum?
Pyoderma gangrenosum is a condition that causes painful skin ulcers and it belongs to a group of skin conditions called neutrophilic dermatoses. Despite the similar name, it should not be mistaken for pyogenic granuloma, which is a totally different condition, even though the names may suggest otherwise. Contrary to what its name might suggest, pyoderma gangrenosum is not a result of infection or gangrene, which means tissue death often caused by lack of blood supply. On the other hand, pyogenic granuloma is often linked to other diseases that affect the entire body. The diagnosis of pyoderma gangrenosum is made by checking the symptoms and ruling out other skin conditions that look similar.
What Causes Pyoderma Gangrenosum?
Pyoderma gangrenosum, a painful skin condition, often occurs alongside other health issues. Some of the most frequent ones are rheumatoid arthritis (a chronic inflammatory disorder affecting joints), inflammatory bowel disease (conditions affecting the digestive tract), and other conditions related to the immune system or inflammation. In addition, pyoderma gangrenosum is linked with solid tumors and blood cancer (hematologic malignancies).
Research has shown that pyoderma gangrenosum coincides with ulcerative colitis (a form of inflammatory bowel disease causing inflammation and sores in the colon) in 5% to 12% of cases, and with Crohn’s disease (another type of inflammatory bowel disease that can affect any part of the digestive tract) in 1% to 2% of cases. However, it’s unclear whether the presence of pyoderma gangrenosum is related to the severity or occurrence of inflammatory bowel disease flare-ups.
Interestingly, treating the associated bowel disease doesn’t always solve the issue of pyoderma gangrenosum. A study of 103 patients with pyoderma gangrenosum revealed that 20% of the patients had blood disorders and 19% had arthritis that couldn’t be detected through blood tests (seronegative arthritis).
Risk Factors and Frequency for Pyoderma Gangrenosum
The occurrence of this disease in children is quite rare, with estimates suggesting it happens in less than 5% of cases. People can develop the disease at any age, usually between 11 and 89 years.
Signs and Symptoms of Pyoderma Gangrenosum
Pyoderma gangrenosum is a condition that has different clinical forms, including vesicular-bullous, pustular, ulcerative, and superficial granulomatous. The vesicular-bullous type, which usually appears on upper body parts and face, is similar to another skin disorder called Sweet syndrome and might cause symptoms like fever and joint pain. A variant called pustular pyoderma gangrenosum is characterized by many small pustules, which might heal or turn into ulcers and is often linked with inflammatory bowel disease.
Beyond these types, another form, called the vegetative form, can show up as well. Some less common areas that can be affected by the condition, such as the vulva and peristomal area, can be hard to treat. Patients with pyoderma gangrenosum often feel pain related to their sores, and the intensity doesn’t always correlate with the sore’s size. When the condition’s sores heal, they typically leave a specific type of scar called a cribriform scar.
Pyoderma gangrenosum has a special feature known as pathergy. This term refers to the skin’s overly aggressive reaction to minor injuries or the worsening of an existing wound due to slight harm or trauma. This reaction can complicate the patient’s routine wound care and make the decision for surgeries or procedures trickier. Even simple skin injuries that generally don’t cause any issues could result in severe complications for pyoderma gangrenosum patients.
Testing for Pyoderma Gangrenosum
Pyoderma gangrenosum is a medical condition diagnosed by ruling out other conditions, both with physical check-ups and lab tests. The biological markers in lab tests for this condition aren’t specific, making the diagnosis a bit challenging.
In 2004, some guidelines for diagnosing this condition were proposed, but they haven’t been confirmed. Based on these guidelines, there have to be two main symptoms and at least two smaller signs to confirm the diagnosis.
Main symptoms include:
– Quick development of a painful, damaging skin ulcer with uneven edges that are purple and look like they’ve been eroded.
– No other possible causes of skin sores are present.
Smaller signs include:
– Patient’s medical history indicates a possible tendency to develop new sores when the skin is injured or scarred in a pattern similar to a sieve (known as pathergy or cribriform scarring).
– Presence of other conditions that commonly occur along with pyoderma gangrenosum.
– Lab test results showing specific types of inflammation in the skin, which are usually not infected by bacteria.
– The sores improve quickly when treated with systemic steroids, a type of medication.
There are several health conditions where pyoderma gangrenosum is considered one of the main symptoms. These include PAPA syndrome (includes pyoderma gangrenosum, acne, and a certain kind of non-bacterial arthritis) and PASH syndrome (includes pyoderma gangrenosum, acne, and hidradenitis suppurativa, a skin disease that causes painful bumps under the skin).
A good way to differentiate between these disorders is to remember that all of them involve pyoderma gangrenosum and acne as symptoms, but differ based on the presence of other conditions like hidradenitis suppurativa, colitis (a type of intestinal inflammation), and certain kinds of arthritis. These conditions all share a common characteristic: they involve disruption of the immune system, specifically with a protein called interleukin-1.
The pathergy skin test is when the skin is pricked to check for an overly strong skin reaction.
Treatment Options for Pyoderma Gangrenosum
When dealing with a condition like pyoderma gangrenosum, which is a rare skin disorder causing painful sores or ulcers, addressing any other existing health problems is critical. However, there isn’t a clear connection between the severity of the skin condition and the seriousness of other health issues.
How fast to act with further treatment largely depends on how quickly the skin condition is worsening. If the affected area starts to increase rapidly in size, more intensive treatments, such as medications (systemic medications) that slow down your immune system may be required. These could be corticosteroids or cyclosporine, for instance. A study done in 2015 found that both these medications were effective, with about 47% of patients experiencing healed ulcers after six months, regardless of the drug used. However, some severe side effects like infections were more commonly seen in patients using prednisolone.
If a patient has previously suffered from pyoderma gangrenosum, especially an aggressive form of it, some doctors may opt to use immune system-suppressing drugs ahead of a surgery. The purpose here is to mitigate the risk of the skin condition returning post-surgery. A typical approach might include using a combination of methylprednisolone and cyclosporine. As a general rule, avoiding unnecessary surgeries or procedures is essential in managing this skin condition.
For less severe or limited instances of this disease, treatments applied directly to the skin (topical therapies) may be adequate. Topical steroids and a medication called tacrolimus have been found effective in such cases. Other treatments that can be helpful include nicotine, a medication called dapsone, and sodium cromoglycate.
Important aspects of managing pyoderma gangrenosum include pain control and proper wound care. Both patients and healthcare providers need to work together to keep the wound clean and prevent infection. While getting rid of dead or damaged tissue (debridement) around the wound is important, care must be taken as unnecessary trauma can trigger a new lesion, a phenomenon known as pathergy.
In addition to the aforementioned therapies, some other treatments have also proven successful. These include drugs like etanercept and adalimumab, which block a substance your body makes called TNF-alpha. A medication called ustekinumab, which is often used to treat psoriasis, a common skin condition that causes skin cells to build up and form scales and itchy, dry patches, has also been found to improve pyoderma gangrenosum. Other potentially effective treatments include Canakinumab and tocilizumab, which are antibodies that target and block the effects of certain substances in the body that are involved in inflammation.
What else can Pyoderma Gangrenosum be?
When a doctor is trying to figure out the cause of a patient’s condition, they consider different diseases that could explain the symptoms. This is called a differential diagnosis. In this case, they might consider:
- Infectious diseases, like certain types of bacterial or fungal infections.
- Non-infectious diseases, such as iododerma or bromoderma, which are skin conditions often caused by exposure to iodine or bromine.
- Other types of ulcers, including arterial ulcers and Martorell ulcers. An arterial ulcer is a sore on the skin that forms when an artery doesn’t effectively supply blood to the skin. A Martorell ulcer is a type of ulcer that usually occurs on the lower leg.
For example, when it comes to arterial ulcers, the doctor might notice that the patient has weak pulses on the same side of the body where the ulcer is located.
