A 59-year-old male patient presented with recurrent erythema and wheals all over the body for 6 years, complicated by irregular fever (the highest body temperature T:p.T221, c.732:G > A:p.A244A and c.786:A > G:p.R262R. Finally, the patient was diagnosed with Schnitzler′s syndrome. There was no improvement of symptoms after the treatment with multiple oral antihistamines at increased dose levels. Then, the treatment protocol was adjusted to oral ciclosporin at a dosage of 200 mg/d for consecutive 18 days, but the patient still showed no response. After the treatment with oral prednisone (30 mg/d) and Tripterygium wilfordii tablets (66 μg thrice a day) for 2 weeks, the skin rashes subsided gradually, and arthralgia was relieved. Moreover, the WBC count, ESR and CRP level were decreased to 9.01 × 109/L, 50mm/1 h and 6 mg/L respectively, while the hemoglobin level was increased to 109.7 g/L. After 1-year follow-up, the dosage of glucocorticoids was gradually decreased to 8 mg/d. In addition, his condition was controlled well with no skin lesions and fever, except for occasional arthralgia in the knees and hip.
Key words:
Schnitzler syndrome; Urticaria; NLRP3