Refractory pemphigus vulgaris successfully treated with Ofat
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A 34-year-old woman, with active mucocutaneous PV disease diagnosed at age 25, did not achieve remission with doxycycline, high-dose prednisone, mycophenolate mofetil (MMF), and methotrexate. Ten days after her first rituximab infusion, bilateral progressive lower extremity weakness developed, resulting in hospitalization.

Over the next 18 months, the patient's disease remained intermittently controlled on prednisone, MMF, and IV(Ig). However, when the patient was discovered to be 8 weeks pregnant, given teratogenicity, MMF was discontinued, and IVIg monotherapy was initiated. Following delivery of a healthy child, the combination regimen of prednisone, 30 mg daily, MMF, 1500 mg BD, and IVIg monthly was restarted.

Over the next 7 months the patient continued to have severe oral mucosal involvement and innumerable flaccid bullae and erosions on her trunk and extremities, which prompted administration of several high-dose pulses of IV methylprednisolone (1 mg/kg for 3 days), an increase in her prednisone to 60 mg daily, and an increase in the frequency of IVIg infusions to every 3 weeks. After 1 month, the patient was hospitalized for lower extremity edema and tachycardia secondary to multiple acute pulmonary emboli.

The care team elected to trial off-label treatment with Ofatumumab (300 mg on day 1, 1000 mg on day 8, then 8 cycles of 1000 mg every 28 days). Patient experienced clinical improvement after her 2nd infusion, and following her 4th infusion, her prednisone was tapered below 20 mg for the first time since initial diagnosis. Patient achieved complete clinical resolution 9 months after initiation of ofatumumab.