Rjm. Engler et al., CHRONIC SULFASALAZINE THERAPY IN THE TREATMENT OF DELAYED PRESSURE URTICARIA AND ANGIOEDEMA, Annals of allergy, asthma, & immunology, 74(2), 1995, pp. 155-159
Background: Delayed pressure urticaria/angioedema can be profoundly di
sabling with painful and prolonged swelling of feet and hands as well
as systemic symptoms of malaise and flu-like illness. Occupations requ
iring prolonged standing and forceful use of hands may be seriously co
mpromised by this condition. The severe forms of the disease are usual
ly unresponsive to antihistamines and nonsteroidal anti-inflammatory d
rugs, and patients frequently require corticosteroids for control of s
ymptoms. Objective: It was the purpose of this report to evaluate the
clinical utility of sulfasalazine for two patients with refractory del
ayed pressure urticaria. Methods: Sulfasalazine, starting at 500 mg/da
y (with weekly incremental dosing to a total of 4 g), was administered
to two patients with disabling pressure urticaria and angioedema (sym
ptomatic daily with normal activities) who had failed all other report
ed therapeutic options except corticosteroids. Results: Patient A requ
ired daily prednisone in excess of 30 mg (for more than 6 months) to c
ontrol his painful angioedema sufficiently in order to continue workin
g asa colorectal surgeon. Patient B also experienced daily symptoms fo
r 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 star
ting but must maintain a dose of 2 g or greater per day. Patient B rep
orted excellent control 6 months after starting but was subsequently l
ost to follow-up. Conclusion: Sulfasalazine, in doses used for inflamm
atory bowel disease, appears to be an effective alternative therapy fo
r delayed pressure urticaria and angioedema in patients poorly control
led by traditional treatment and may act as a corticosteroid-sparing a
gent.