Pyoderma gangrenosum is a rare but serious ulcerating skin disease causing painful ulcers on the legs and other parts of the body. Diagnosis of the condition is difficult due to a lack of biomarkers or other distinguishing features, and treatment can also be challenging. No single specific therapy for pyoderma gangrenosum is available and few clinical trials have been performed into the condition.

Pyoderma gangrenosum is generally managed through a stepwise approach using a combination of systemic and topical therapies. Immunosuppression with corticosteroids and ciclosporin is the mainstay of treatment, with the use of other agents largely dependent on local protocols and experience.(1-3)

Topical Treatments

Topical treatment is essential for the management of emerging ulcers. Highly potent topical corticosteroids have been shown to induce remission in certain cases, while triamcinolone injected into the ulcer edge and topical tacrolimus have both been shown to be effective certain patients.(1) Since most ulcers present with heavy exudate, foam or multi-layered laminate dressings are generally recommended. Pain and/or odor may sometimes be relieved using a compress.(2)  

Systemic Treatments

Systemic therapy is particularly important in patients with widespread or rapidly progressing disease. Most patients are prescribed oral corticosteroids early in the disease to try to induce remission. Patients receiving long-term steroid therapy may also require a bone-protecting agent such as minoscycline. The other widely used immunosuppressant is ciclosporin, which can reduce dependence on corticosteroids. Good responses can be achieved with ciclosporin, but side effects including nephrotoxicity, hypertension and an increased risk of cancer have been reported. Other systemic agents that show some benefit include azathioprine and tacrolimus, while anti-tumor necrosis factor-α agents may also induce some response.(1)

In extreme cases, surgery may be used, although this can itself be a trigger for pyoderma gangrenosum.(2) Autologous split-skin grafts have been used with some success, although this necessitates the creation of a new wound at the donor site. Use of bioengineered skin may be an option in the future.

Treatment of pyoderma gangrenosum is often based on trial and error, and long-term outcomes are unpredictable. However, if poorly managed, the condition can be severely debilitating with a devastating effect on quality of life. Continued professional training can provide the opportunity to study rare but complex conditions such as pyoderma gangrenosum in greater depth. For more information on the benefits of wound care certification, please visit www.woundeducators.com.

References

1.         Brooklyn T, Dunnill G, Probert C. Diagnosis and treatment of pyoderma gangrenosum. BMJ. 2006;333:181-4.

2.         Wollina U. Pyoderma gangrenosum--a review. Orphanet J Rare Dis. 200715;2:19.

3.         Jackson M, Callen JP. Pyoderma gangrenosum.  Medscape March 2010 (available at http://emedicine.medscape.com/article/1123821-overview#a0199).