Rare disorder causes chronic skin condition

DEAR DR. GOTT: I have an open wound on my leg that my surgeon thought was an infection. After eight surgeries, I was eventually diagnosed (by a dermatologist) with a rare immune-system disorder called pyoderma gangrenosum. I still have the open wound after two years. It is healing very slowly. Please tell me what you know about this condition.

DEAR READER: Pyoderma gangrenosum is a rare skin condition that causes slow-healing, painful ulcers to form, typically on the legs. The cause is not currently known but is thought to be an abnormal immune response. About half of all sufferers have an underlying immune disorder. Health conditions that may be related include rheumatoid arthritis, lupus, vasculitis, leukemia, sarcoidosis, hepatitis and, particularly, inflammatory-bowel diseases, such as ulcerative colitis and Crohn’s disease.

Lesions typically start as one or more small, red bumps that can resemble a spider bite that progresses to become a painful, open wound with a reddish-purple border. Other symptoms include a general feeling of poor health, achy joints and bone pain. Scarring is common.

If an underlying health condition is associated with pyoderma gangrenosum, treating it may help control the sores, but often direct treatment of the ulcers will also be necessary. The primary course of action is oral steroids, which reduce inflammation and somewhat suppress the immune system. Mild cases may require only topical steroids. Because side effects are common, it is important to use the lowest dosage that provides results for the shortest period of time. Other drugs include immunosuppressant medications, tumor necrosis factor inhibitors, nonsteroidal anti-inflammatory drugs (NSAIDs) and high-dose intravenous immunoglobin.

Surgery isn’t commonly considered an option because skin trauma, including surgical incisions, may worsen existing lesions and/or cause new ones to develop. In cases of severe ulceration, skin grafting or debriding (cleaning away dead skin/tissue) can be beneficial but must be done carefully, preferably by a skilled surgeon familiar with pyoderma gangrenosum.

With treatment, healing typically occurs within several months. Without it, the sores may remain the same, worsen or heal even more slowly than those with treatment.

Sufferers should be gentle with their skin and take care to avoid injury. It is important to follow your physician’s care instructions in order to speed healing and reduce scarring.