Autoimmune Aspects of Pemphigus Vulgaris and Mucosal Pemphigoid

Abstract

Oral mucosal lesions of ulcerative, desquamative, and bullous character are major problems for clinicians and patients alike. Underlying such processes is the autoimmune reaction manifesting as specific subsets of immunopathology. Responsible for the maintenance of mucosal integrity within the oral cavity are numerous adhesion proteins which operate at the cell-to-cell and cell-to-connective-tissue-matrix level. Two of the better-understood conditions characterized by altered or disordered levels of adherence are the pemphigus and the pemphigoid groups of lesions. Autoantibodies in pemphigus vulgaris specifically attack normal proteins within the desmosomal structure. Cellular adhesion is affected by steric interference by antibodies directed against antigens residing within the intercellular space or the desmosome itself. Such alterations ultimately result in cell-to-cell separation, with production of the acantholytic element characteristic of pemphigus vulgaris. Mucous membrane pemphigoid is characterized by a separation at the junction of the epithelium and connective tissue at the level of the basement membrane complex. Histologically, a split occurs in the sub-basilar region, with antibodies directed to the 230-kDa protein component of the hemidesmosome. Minor antigens residing within the basement membrane area include a 180-kDa element as well as other less commonly found proteins near the membrane complex. Manifestations of these illnesses are often severe. Management strategies of pemphigus vulgaris have changed over the recent past, with new modalities supplementing the traditional corticosteroid approach. Both conditions are usually lifelong, with the implications of management a major consideration.

Clinical features of gingival pemphigus vulgaris

“…This ultimately results in cell to cell separation, with production of acantholysis (Eversole 1994, Amagai et al 1991, Wilkinson et al 1989). DIS analysis shows predominantely IgG, and sometimes IgG plus C 3 , IgA, and/or IgM deposited in the intercellular space in either the lesional epithelium or of clinically normal epithelium adjacent to the lesions (Eversole 1994, Sciubba 1996).…”

The management of oral mucous membrane pemphigoid with dapsone and topical corticosteroid

“…Dapsone blocks PMN migration, thereby preventing inflammation or new vesicle formation (13). Dapsone therapy is not without adverse effects; however, clinicians using dapsone must be familiar with its potential side effects.…”

The Management of Oral Pemphigus Vulgaris with Systemic Corticosteroid and Dapsone

“…17 Dapsone blocks PMN migration, thereby preventing inflammation or new vesicle formation. 18 Dapsone toxicity can be categorized as either idiosyncratic or dose-dependent. 19 Long-term administration in normal patients may result in significant methemoglobinemia.…”