Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease in which pathogenic autoantibodies preferentially target the noncollagenous 16A domain of collagen XVII present in hemidesmosomes. 1 Rituximab has proven effective for the treatment of steroid-refractory BP, 2 but there is controversy over the optimum dosing protocol. Here we report three cases of elderly BP patients treated with an ultra-low dosage (100 mg weekly for 4 weeks) of rituximab successfully.
| C A S E REP ORTSPatient 1, a 63-year-old male, presented with erythema, bulla, and erosions on his trunk and extremities with significant itching for 3 months. Physical examination revealed tense bullae and erosions on the extremities and trunk (Figures 1a,b). Eosinophil absolute value was 1.82 × 10 9 /L (normal value <0.52 × 10 9 /L), total IgE was 2500 IU/ml (normal value <200 IU/ml), anti-BP180 antibody was 248.77 U/ml (normal value <9 U/ml), and anti-BP230 antibody was 1.11 U/ml (normal value <9 U/ml). Skin biopsy from the arm revealed subepidermal blister formation with eosinophilic and lymphocytic infiltration in the upper dermis (Figure S1a). Direct immunofluorescence (DIF) showed linear deposition of IgG and C3 at the basement membrane zone; IgA and IgM were not detected. A diagnosis of BP was made.The patient was treated with oral prednisone 0.75 mg/kg once a day and oral methotrexate 10 mg once a week, together with topical corticosteroid. Pruritus was soon relieved and the skin lesions slowly resolved in 2 weeks. Two more weeks later, when the oral prednisolone dose was tapered to 0.5 mg/kg daily, new bullae developed all over his body again. Then he was treated with ultra-low-dose rituximab injection (100 mg) intravenously once a week for 4 weeks,