A 29-year-old female presented with a 5 year history of pemphigus, characterized by recurrent painful sores in her mouth. She denied ever having lesions elsewhere on her body. Physical examination showed erosive or desquamative gingivitis of the upper and lower anterior and lateral gingival surfaces. Serologic studies revealed moderate to high anti-desmoglein 1 titers, ranging from 28 to 54 over the course of her disease, while anti-desmoglein three levels were always within normal limits. The patient was previously diagnosed with limited oral pemphigus based on routine histology, direct immunofluorescence findings and anti-desmoglein 1/3 ELISAs. Over the years, she had partial disease control with a variety of agents including: topical steroids, topical tacrolimus, topical dapsone and systemic prednisone, mycophenolate mofetil, and a combination of doxycycline + niacinamide. Hydroxychloroquine, dapsone and adalimumab were each tried for 3–6 months without efficacy. In addition to having recalcitrant pemphigus, she expressed interest in wanting to become pregnant.

Therapeutic Challenge: To find an efficacious medication that is safe during conception and pregnancy.

Based on the above case, which of the following medications would be a reasonable therapy in a female of childbearing age with resistant pemphigus who wants to get pregnant?

Answer: (A)

The patient was started on prednisone 20 mg/day and auranofin 3 mg three times daily combined with intermittent topical intraoral steroids for transient lesions. She had complete clearing of her oral erosions over 3 months and was tapered to 3–6 mg/day prednisone, subsequently conceived while on auranofin and continued on a dosage of auranofin 3 mg twice daily throughout her pregnancy. Desmoglein 1 antibodies ranged from 32 to 39 and desmoglein 3 antibodies remained negative during an uneventful pregnancy, resulting in a healthy, full-term baby. Approximately 9 months postpartum she began to flare despite increased oral steroids back to 20 mg/day and auranofin 3 mg three times daily. Desmoglein 1 antibody levels had risen to 49. She is currently tapering from high dose prednisone (1 mg/kg/day × 4 weeks) and is considering rituximab and/or intravenous gamma globulin as she has completed her family planning and is not planning on any more children.