1 in every 100,000 people in the U.S. has pyoderma gangrenosum. Pyoderma can look like a variety of conditions and can delay proper diagnosis and treatment. Knowing the signs and risk factors can help those who suffer from it avoid serious complications. Although the exact cause is unknown, clinical research is paving the way for prevention and treatment options.
Definition
Pyoderma gangrenosum (pie-o-DUR-muh gang-ruh-NO-sum) is a rare, severe condition that causes large and painful ulcers to develop on your skin. These ulcers can develop anywhere on the skin but are most often found on the legs. Symptoms start with small, red bumps on the skin that can resemble a spider bite. After a few days, the bumps erode and develop into large open sores that are painful. The size and depth of the ulcerations vary, but can be classified into four variations:
- Classic– Mostly occurs on the legs but can also happen near surgical openings like a stoma.
- Atypical/Bullous– This Typically affects the hands and is frequently associated with an underlying condition, especially hematological malignancy such as leukemia.
- Pustular– This variation is usually found on the arms and legs and is often associated with inflammatory bowel disease.
- Vegetative– Vegetative PG is characterized by ulcerations that are chronic, but not painful.
The cause of PG remains unknown; however, it is suspected to be an autoimmune disease. Around 50% of cases are associated with other underlying disorders, especially Crohn’s Disease, inflammatory bowel disease, and ulcerative colitis. It affects women a little more often than men and typically occurs between the ages of 20-50.
Diagnosis and Treatment
There is no specific test to diagnose PG. Diagnosis will take a variety of tests and the patient’s medical history to rule out other conditions. Also, a skin biopsy is often performed.
Treatment will depend on the number, size, and depth of your ulcerations, and may require multiple approaches. Options include open, wet dressings on ulcers, topical anti-inflammatories, injectable corticosteroids, anti-bacterial agents, and immunosuppressive therapies. The skin must also be protected from further injury, which could result in new ulcerations. In some cases, skin grafts may be needed once inflammation is under control.
Clinical Research and Treatment Options for PG
Potential treatments through clinical research are being explored. These involve the drugs tacrolimus, ustekinumab, chlorambucil, mycophenolate mofetil, and intravenous immune globulin. Additional research is needed to validate the safety and effectiveness of these treatments long term for those with PG.
If you or a loved one is suffering from PG, clinical research studies may be an option. Qualified participants receive access to study-related treatments and care and reimbursement for time and travel. To learn more about our currently enrolling studies for PG, call (813) 264-2155.
References:
https://rarediseases.org/rare-diseases/pyoderma-gangrenosum/
https://www.mayoclinic.org/diseases-conditions/pyoderma-gangrenosum/symptoms-causes/syc-20350386
