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Pemphigus vulgaris is an autoimmune bullous disease affecting the skin and mucosa. It is caused by autoantibodies targeting the adhesion molecules desmoglein 1 and desmoglein 3, which, respectively, mediate adhesion between keratinocytes in skin and mucosa, resulting in the formation of blisters and erosions. Early recognition and treatment are crucial for remission of this disorder. Treatment for severe pemphigus traditionally includes long-term corticosteroids and immunosuppressants [1]. Rituximab, a chimeric monoclonal antibody against the CD20 antigen of B lymphocytes, was recently introduced and has proven to be effective in resistant and life-threatening pemphigus [2]. Protocols for administration of rituximab are determined for use in nonHodgkin lymphoma (375 mg/m2 once a week, with duration depending on the type of lymphoma) and for rheumatoid arthritis (2 doses of 1000 mg given 2 weeks apart) [3]. A lower dose of rituximab (500 mg at 2 weeks interval) can be used in pemphigus [4-6]. A 67-year-old woman was referred to our dermatology department for recent onset of erosive lesions of the oral cavity. The patient complained of severe difficulty swallowing, forcing her to feed almost exclusively with a liquid diet. The skin was not involved, showing only signs of previously active lesions. She denied trauma and application of topical drugs on the areas involved. Her medical history did not reveal any association with other immune-mediated diseases. Evaluation of circulating anti-DSG1, anti-DSG3, and antiBP180 antibodies revealed a high anti-DSG3 autoantibody titer (192.13 U/mL, normal value <7 U/mL), which is consistent with a diagnosis of pemphigus vulgaris. Treatment was initially started with intravenous methylprednisolone 80 mg/d for 2 weeks, which was thereafter slowly tapered to 40 mg/d over 2 weeks, with complete remission. The patient was then treated with oral prednisone 50 mg/d for 1 month and 25 mg/d for a further month. At this time, the disease relapsed, with oral involvement only, and azathioprine 100 mg/d was combined with prednisone 25 mg/d for 3 months. The oral lesions did not resolve. Consequently, we decided to administer rituximab [7] in a 2-dose regimen (2 weeks apart) of 500 mg per dose. Prior to infusion of rituximab, the patient was premedicated with intravenous acetaminophen 1000 mg, chlorphenamine 10 mg, and methylprednisolone 20 mg diluted in 100 mL of normal saline. Rituximab 500 mg was then diluted in 500 mL of 347

Persistent B Lymphocyte Depletion After an Ultralow Dose of Rituximab for Pemphigus Vulgaris