British fournal of Dermatology 1995: 132: 824-826. Successful treatment of epidermolysis bullosa acquisita using intravenous immunoglobulins C.MOHR. C.SUNDERKOTTER. A.HILDEBRAND. K.BIEL. A.RUTTER. G.H.RUTTER.' T.A.LUGER AND G.KOLDE Department of Dermatology. Westfalische-Wilhelms-Universitat. von-Esmarch-Str. 56, D-48149 Munster. Germany *DeGab, MUnster. Germany Accepted for publication 17 August 1994 Summary We report a 55-year-old man with severe inflammatory epidermolysis bullosa acquisita. The skin lesions did not respond to various immunosuppressive treatments. The combined administration of prednisone. azathioprine, dapsone and colchicine resulted only in a transient and incomplete resolution of the lesions. The bullae and increased skin fragility were successfully controlled by the addition of high-dose intravenous immunoglobulin therapy. Epidermolysis hullosa acquisita (EBA) is a chronic bullous disease associated with autoantibodies against the carboxyl terminus of type VII collagen within the anchoring flbrils of the dermal-epidermal junctional zone. ' Affected individuals suffer from mechanically induced subepidermal blisters, similar to hereditary dystrophic epidermolysis bullosa.' Clinically, a 'classi- cal' form with scarring and milia is distinguished from a rare inflammatory form mimicking either bullous or cicatricial pemphigoid.*'^ We report a patient with bullous pemphigoid-like EBA, whose disease was resis- tant to various immunosuppressive treatments, but was finally controlled by high-dose intravenous immunoglo- bulins administered in combination with prednisone. azathioprine, dapsone and colchicine. Case report A 55-year-old man with type II diabetes mellitus developed several tense blisters on erythematous skin in the axillae. The bullae subsequently spread over the whole body, including the oral and genital mucous membranes, but the hands and feet were spared. After 9 months of blistering, most of the lesions were found in mechanically stressed skin areas, such as the hands and feet, elbows, knees, shoulders and back (Eig. la). The lesions healed without scarring or milia formation, and the nails were not affected. Histology of skin biopsies showed subepidermal blis- ters, and a dermal inflammatory infiltrate of eosinophils. Direct immunofluorescence demonstrated linear deposits of IgG and complement C3 along the dermal-epidermal junctional zone. Indirect immunofluorescence. using guinea-pig oesophagus as substrate, revealed circulat- ing IgG antibasement membrane zone antibodies in a titre of 1 : 500. A diagnosis of inflammatory EBA was confirmed by electron microscopy, which showed blister formation beneath the junctional basement membrane, and by direct immunofluorescence on salt-split skln,^ which showed that the circulating IgG antibodies were deposited on the dermal side of separated normal human skin. Laboratory investigations revealed an elevated ery- throcyte sedimentation rate (30mm/h; normal <15}. and a normochromic anaemia (haemoglobin 9-9g/dl. normal 14-18; haematocrit 30-2%. normal 42-52). Serum glucose levels ranged from 100 to 180 mg/dl During a 3~year period, several therapeutic regimens, including dapsone (50mg/day). cyclosporin (7mg/kg/ day), and cycophosphamide/dexamethasone pulse therapy,'^ failed to reduce the blister formation and skin fragility. Subsequently, combination treatment with prednisone (12mg/day), azathioprine (100 mg/ day), dapsone (lOOmg/day) and colchicine (2mg/ day) was employed. This led to only a transient reduc- tion of blistering, but did not affect skin fragility.** Because of continuing development of bullae. we started intravenous immunoglobulin therapy, using native 7S IgG. The immunoglobulin preparation was given at a daily dose of 400 mg/kg body weight for 5 days, and the infusions were repeated every 4 weeks. The therapy was well tolerated. After three courses of treatment there was a marked reduction of blistering. 824 199S British Association of Dermatologists