Diagnosis and classification of autoimmune blistering diseases

Blistering skin diseases are a group of autoimmune disorders that are characterized by autoantibodies against structural proteins of the epidermis or the dermal-epidermal junction and clinically by blisters and erosions on skin and/or mucous membranes. Since clinical criteria and histopathological characteristics are not sufficient for diagnosis, direct immunofluorescence microscopy of a biopsy specimen or serological tests are needed for exact diagnosis. The differentiation between the various disorders became more important since prognosis as well as different treatment options are nowadays available for the various diseases. Moreover, some bullous diseases may indicate the presence of an underlying malignancy. The detection of serum autoantibodies have been shown to correlate with disease activity and thus may be helpful in deciding treatment options for these patients.