Pyoderma Gangrenosum
Pyoderma Gangrenosum (PG) is a rare auto-inflammatory skin condition that causes painful, rapidly growing ulcers. It affects approximately six people per million each year. Genetic factors, neutrophil dysfunction, and abnormal inflammation are associated with the condition.
PG can be difficult to diagnose, and for many it has taken a year or more. PG symptoms may resemble those of other conditions, and more than 40% of cases have reportedly been diagnosed only after multiple misdiagnoses. Delayed or incorrect diagnosis can make the condition worse, prolong suffering and contribute to additional healthcare visits, hospitalizations, and time away from work.
Early diagnosis and treatment is absolutely critical to avoid the devestaing consquences of misdiagnosis and mistreatment that could lead to increased pain, years of decreased function, mobility issues, amputation or ever death.
Symptoms and course
The course of PG is unpredictable, and treatment is often by trial and error. Patients may need to try multiple therapies before their wounds heal, and healing has been reported to take around 12 months for many patients. For others it can be months or many years. PG can also leave deep lasting scars. Even after a wound has healed, PG may return to reopen old wounds or create new ones. There is no cure for PG, only treatment.
The impact may extend beyond the ulcers. Pain, wound care, treatment costs, reduced mobility, and changes to work and relationships can affect a person’s daily life and overall well-being. No two people are alike, which makes it difficult for doctors and wound care specialists to treat. What works on one may not work on another. Even for the same person, one wound may respond to a certain drug or treat, and the next wound does not, and you need to start over and find what works for the new wound. PG has a mind of it’s own.
Associated conditions
Around half of PG cases are associated with other conditions, including:
- Inflammatory bowel disease, such as Crohn’s disease and ulcerative colitis
- Inflammatory arthritis, such as rheumatoid arthritis
- Autoimmune diseases, such as systemic lupus erythematosus
- Inflammatory diseases, such as hidradenitis suppurativa and vasculitis
- Hematological disorders
Challenges finding doctors
Because PG is rare and can be difficult to distinguish from other conditions, patients may struggle to find providers familiar with it. Delayed diagnosis may lead to more emergency department visits and hospitalizations and all the costs associated with health care. Even in large cities it can be difficult, or even impossible, to find a doctor who as treated PG before. Many wound care clinics believe a PG ulcer to be a diabetic ulcer and will debride the wound, which is the exact opposite of what you do if its PG. PG responds to pathergy, and some wound care centers will make it worse.
Accessing specialized care can also be challenging. People in underserved communities may face barriers involving internet access, technology, distance, or age-related needs. Travel can be difficult because of work, childcare, mobility, or financial concerns. One report notes that around 39% of patients with rare diseases travel 60 miles to receive medical care.
Improving awareness and access to knowledgeable providers can help address these challenges.
Challenges finding drugs
There are no FDA approved drugs for PG. Treatment typically involves supressing the immune system. The first line of defense is usually high dose steroids, like Prednisone. If that doesn’t work well enough, Cyclosporine may be presribed, or a biologic like Humira or Remicade. In most cases insurance companies deny the drugs that mmight actually work, because PG is considered off-label. That is true with many rare diseases, and is a fault in the current system. Insurance companies are quick to deny anything off-lable, even using it as an excuse.
Doctors try to work around that by finding another condition you may have that requires a biologic, if that condition exists. Even then, insurance companies attempt to deny but often approve on appeal. This makes it challenging for both doctors and patients. The largest benefit to a new PG wound is to attack it early and quickly with powerful medication. A new wound develops rapidly, within a week, and once it goes deep can last years. The key is early diagnosis and treatment, and waiting for insurance appeals usually means its too late and the damage is already done.
It is our hope that current clinical trials will produce an effective drug specially for PG.