Epidermolysis bullosa acquisita treated with basiliximab, an interleukin-2 receptor antibody.

A 55-year-old white man presented with a 2-month history of painful blisters. Tense bullae, erosions and haemorrhagic crusts were distributed on both arms, both legs and the trunk. Nikolsky’s sign was negative. A biopsy specimen from perilesional skin showed haemorrhagic spongiosis of the epidermis, discontinuous subepidermal split formation and a super® cial perivascular in® ltrate composed predominantly of neutrophils. Direct immuno ̄ uorescence was positive for linear IgG, C3 and ® brinogen along the epidermaldermal junction. Western immunoblotting of serum con® rmed autoantibodies against a 290 kDa band corresponding to collagen VII. Prednisolone 100 mg/day was given initially for 10 days but new blisters appeared. Combination therapy with prednisolone 100 mg/ day and azathioprine 100 mg/day for 4 weeks followed by prednisolone 80 mg/day plus cyclosporin A 200 mg/day for a further 5 weeks did not reduce disease activity. Ten weeks after the initial treatment, the patient additionally received an intravenous infusion of 20 mg basiliximab (Simulect; Novartis Pharma, Germany), which was well tolerated without any side effects. Cyclosporin A 200 mg/day was continued and prednisolone was subsequently tapered to 10 mg/ day. Eruption of new blisters stopped within 2 weeks after the infusion of basiliximab and resolution of residual blisters, erosions and crusts was complete within 10 weeks, with partial atrophy and formation of milia.