%0 Journal Article
%T Bullous Skin Diseases: Classical Types of Autoimmune Diseases
%A Jan Damoiseaux
%J Scientifica
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/457982
%X The prototypic bullous skin diseases, pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid, are characterized by the blister formation in the skin and/or oral mucosa in combination with circulating and deposited autoantibodies reactive with (hemi)desmosomes. Koch¡¯s postulates, adapted for autoimmune diseases, were applied on these skin diseases. It appears that all adapted Koch¡¯s postulates are fulfilled, and, therefore, these bullous skin diseases are to be considered classical autoimmune diseases within the wide and expanding spectrum of autoimmune diseases. 1. Introduction The bullous skin diseases, including pemphigus and bullous pemphigoid, affect the skin and/or oral mucosa. Since the skin is a vital organ in the protection of the body against dehydration and infections, these skin diseases may be life threatening. The bullous skin diseases are being divided in two categories based on whether the skin is affected within the epidermis or at the epidermal-dermal interphase. The first category is referred to as pemphigus and entails 4 disease entities: pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and IgA pemphigus. Altogether, the yearly incidence of this category is about 0.3/100,000 and the age of onset of these diseases is primarily in the fifties and sixties. The second category entails multiple disease entities. When considering dermatitis herpetiformis, a bullous skin manifestation of celiac disease, as a distinct subcategory of the pemphigoid skin diseases, the overall yearly incidence of the second category is about 1.0/100,000. These diseases typically become manifest at an age >65 years [1¨C3]. The diagnosis of the bullous skin diseases is based on the typical skin manifestations, which may be objectified by the Nikolsky sign and characteristic direct immunofluorescence (DIF) patterns in skin biopsies (Table 1). The presence of skin-specific autoantibodies in the circulation will further add to the diagnosis [1]. Table 1: Clinical and laboratory characteristics of bullous skin diseases. In the current paper Koch¡¯s postulates for defining infectious diseases and adapted for autoimmune diseases are applied on both categories of bullous skin diseases [4, 5]. Since these skin diseases fulfil all criteria, they can be considered to belong to the small number of unequivocal autoimmune diseases within the ever expanding number of diseases that are supposed to be autoimmune. 2. Koch¡¯s Postulates Adapted for Autoimmune Diseases While Koch¡¯s postulates were originally intended to define the infectious origin of a
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