A Rare Case of Autoimmune Progesterone Disease

Background: Autoimmune progesterone disease is a rare disease, with fewer than 200 reported cases. Unfortunately, there are no published estimates of incidence or prevalence. Clinical Case: A 24 year-old woman with no significant past medical history presented with sudden and new onset swelling of the lips which was unresponsive to Benadryl and prednisone. Swelling eventually progressed into blistering over the lips, oral, pharyngeal mucosa and tongue. She was admitted to the hospital for suspicion of Steven Johnson syndrome and was discharged after resolution of skin and oral lesions following treatment. She presented again in 3 months with full body rash and blisters now involving oral and vaginal mucosa. She underwent biopsy of these lesions during this admission and findings were suspicious for drug eruption vs erythema multiforme. Unfortunately, inciting drug or event could not be associated even with pathological diagnosis. She continued to have mucocutaneous flare every month for the next 8 months with multiple hospitalizations and was treated with antibiotics during these admissions. Over the course of her evaluation, it was noted that her symptoms seemed to coincide with her menstrual cycles. Her IUD was removed and she was started on OCPs but her symptoms persisted without any improvement. She underwent intradermal progesterone challenge test at tertiary center for ongoing cyclical dermatitis and tested positive for progesterone sensitivity. She was diagnosed with Autoimmune Progesterone dermatitis. She had to ultimately undergo total abdominal hysterectomy and bilateral salphingo-oophorectomy as she failed OCPs and had adverse effects to progesterone desensitization including full body rash and blisters. Post operatively, she has been started on IM depot injections of estrogen and vaginal estrogen cream. Conclusion: Autoimmune progesterone disease also known as progesterone dermatitis or progesterone hypersensitivity is not associated with other autoimmune diseases and usually affects women of reproductive age. It is a rare disorder with variable presentation and often overlaps with other forms of dermatosis. It is commonly underdiagnosed or misdiagnosed and appropriate treatment is often delayed. High clinical suspicion for symptoms of cyclical nature is necessary for making the diagnosis.