Chronic sulfasalazine therapy in the treatment of delayed pressure urticaria and angioedema

Abstract

Background: Delayed pressure urticaria/angioedema can be profoundly disabling with painful and prolonged swelling of feet and hands as well as systemic symptoms of malaise and flu-like illness. Occupations requiring prolonged standing and forceful use of hands may be seriously compromised by this condition. The severe forms of the disease are usually unresponsive to antihistamines and nonsteroidal anti-inflammatory drugs, and patients frequently require corticosteroids for control of symptoms.

Objective: It was the purpose of this report to evaluate the clinical utility of sulfasalazine for two patients with refractory delayed pressure urticaria.

Methods: Sulfasalazine, starting at 500 mg/day (with weekly incremental dosing to a total of 4 g), was administered to two patients with disabling pressure urticaria and angioedema (symptomatic daily with normal activities) who had failed all other reported therapeutic options except corticosteroids.

Results: Patient A required daily prednisone in excess of 30 mg (for more than 6 months) to control his painful angioedema sufficiently in order to continue working as a colorectal surgeon. Patient B also experienced daily symptoms for more than 1 year. Both patients tolerated sulfasalazine to a dose of 4 g/day without adverse reactions and achieved complete resolution of symptoms. Patient A continued to be well controlled 1 year after starting but must maintain a dose of 2 g or greater per day. Patient B reported excellent control 6 months after starting but was subsequently lost to follow-up.

Conclusion: Sulfasalazine, in doses used for inflammatory bowel disease, appears to be an effective alternative therapy for delayed pressure urticaria and angioedema in patients poorly controlled by traditional treatment and may act as a corticosteroid-sparing agent.