Autoimmune blistering diseases of the skin

Autoimmune skin diseases represent a heterogeneous group of disorders with grossly diverging clinical manifestations but partly shared underlying immunological mechanisms. They may affect the skin as an isolated organ or among systemic diseases. In addition unspecific cutaneous symptoms or drug-induced unwanted effects can be seen and have to be carefully dissected from an exacerbations of the underlying disease. Growing pathogenic knowledge has elucidated serological and clinical pictures heterogeneity and at the same time increased the therapeutic armentarium for these partly life-threatening diseases. In this review, the focus is on autoimmune bullous diseases with the skin as the major target which involve antigens of epidermis, basal membrane or dermis. Among these the pemphigoid and pemphigus group may be differentiated from dermatitis herpetiformis Duhring and epidermolysis bullosa acquisita. Interestingly, pathogenetically relevant antibody responses of IgA subtype can be found in any of the first three groups. Their clinical picture as well as therapeutic response are distinctly different from the other mainly IgG mediated subsets. Though systemic corticosteroids are still the mainstay of therapy, differential approaches using diverse adjuvant drugs are available. Immunoserological data may help characterize subsets and monitor clinical diseases as well as therapeutic response.