Chronic autoimmune urticaria with worsening during the luteal phase suggestive of progesterone hypersensitivity successfully treated with rituximab.

A 30-year-old woman presented with glucocorticoid-dependent chronic spontaneous urticaria (CSU) previously treated with high-dose H1-second generation antihistamines, omalizumab, and cyclosporin (This is the current Chronic spontaneous urticaria guidelines: Step 1 - second generation H1 antihistamine up to 4 times than standard dose; Step 2 - Omalizumab; Step 3 - Cyclosporin) that were either intolerable or ineffective at providing symptom relief. She also had two prolonged treatment courses with omalizumab that failed. Her CSU was cyclical worsening during ovulation and improving but not resolving midway through her cycle. Markers for chronic autoimmune urticaria (CAU) were positive. Treatment with progesterone desensitization was partially successful, which allowed her to taper her daily glucocorticoids to prednisone 20 mg a day. She was diagnosed with progesterone hypersensitivity with concomitant CAU. Subsequent treatment with rituximab to remove pathogenic autoantibodies was successful in allowing her to taper off prednisone and tolerate progesterone without hives during or independent of her menstrual cycle. She subsequently was able to conceive a healthy baby girl without recurrence of hives.