Bullous pemphigoid: a new look at a well-known disease

Abstract

Bullous pemphigoid is an autoimmune blistering disease that is associated with appearance of subepidermal blisters. IgG antibodies against components of the epithelial basement membrane (BP 180 and 230 antigens) can be typically found in serum of patients. Direct immunofluorescence reveals usually a linear deposition of IgG and/or C3 along the basement membrane, but other immunoglobulins may also be present. Our patient had no detectable formation of tense blisters of his skin; instead, the clinical picture was rather compatible with a prurigo simplex subacuta or a pruritic variant of atopic dermatitis. He also had a markedly elevated total serum IgE level. The diagnosis was confirmed by detection of circulating IgG against BP 180 in patient's serum in ELISA and detection of linear IgG deposits along the basement membrane zone of epidermis in the direct immunofluorescence. Atypical clinical presentations of bullous pemphigoid without blister formation are possible. Recent studies show that an increased total IgE level in serum of patients may occur frequently. The aim of this report is to provide the reader with a brief insight in the new findings in pathogenesis and therapy of bullous pemphigoid.