open to eligible people ages 18 years to 110 years
Background: - Inflammatory pustular skin diseases are a type of autoinflammatory disease in which the immune system attacks the body s tissues. These diseases cause painful and itchy skin rashes, eye and mouth irritation, joint pain and fever. Several drugs for treating these diseases suppress the immune system. However, they can cause severe side effects when taken over a long period of time. - IL-1 is a small protein that may be important in causing the inflammation seen in pustular skin disease. Anakinra is a drug that works by blocking IL-1. It has been effective in treating some inflammatory conditions such as rheumatoid arthritis. However, anakinra has not been studied for use in patients with pustular skin disease. Researchers want to see whether anakinra will be effective in treating pustular skin disease. Objectives: - To see if anakinra can be used to treat inflammatory pustular skin disease. Eligibility: - Individuals at least 18 years of age who have inflammatory pustular skin disease. Design: - Participants will be screened with a physical exam and medical history. Their disease will be evaluated with blood tests, urine tests and imaging studies. Skin biopsies may also be collected. - Participants will have an initial visit to receive the first dose of anakinra. They will be shown how to give themselves daily injections of anakinra. - Participants will take anakinra for up to 12 weeks as long as there are no severe side effects. During this time, they will keep a study diary to record the severity of any rashes, pustules, itching, fevers, and skin or joint pain. They will bring this diary to their study visits. - Participants will have study visits at weeks 4, 8 and 12. Treatment will be monitored at these visits with blood tests, urine tests and physical exams. Depending on the effects of the treatment, participants may have the dose of anakinra increased or decreased. - Participants will have a final study visit 4 weeks after they stop taking anakinra.
San Francisco, California and other locations