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Oral Manifestations of Autoimmune Blistering Diseases

Background

Oral lesions are observed commonly in autoimmune blistering skin diseases. Oral lesions can be the predominant or minor clinical manifestation of a given disease. Pemphigus vulgaris (PV) and bullous pemphigoid (BP) are the earliest recognized autoimmune blistering diseases, and, together, they account for about one half of the autoimmune blistering diseases. While most patients with pemphigus vulgaris have oral lesions, which usually are the first manifestation of this disease, only a few patients with bullous pemphigoid have oral lesions. Over the last few decades, many other autoimmune blistering diseases have been delineated, and some of these newly identified diseases have oral manifestations.

This article discusses the oral manifestations of several well-characterized autoimmune blistering diseases, including pemphigus vulgaris, bullous pemphigoid, linear immunoglobulin A (IgA) bullous dermatosis, and paraneoplastic pemphigus (PNP). A group of autoimmune blistering diseases affecting primarily the mucous membranes is termed mucous membrane pemphigoid (MMP) (also termed cicatricial pemphigoid). Because this topic is discussed in a separate article, it is not described in great detail in this article.

Animal models

Spontaneous animal homologues of human autoimmune blistering diseases have been identified in the last 2 decades.
Those diseases in which oral involvement occurs include pemphigus vulgaris (dogs, cats), paraneoplastic pemphigus (dog, cat),
bullous pemphigoid (dogs, cats, horses, pigs),
mucous membrane pemphigoid (dogs, cats),
linear IgA bullous dermatosis (dogs), epidermolysis bullosa acquisita (dogs), and bullous systemic lupus erythematosus (1 dog). The histopathologic and immunopathologic findings usually are the same as that of human diseases and are not discussed here.

Pemphigus group

Pemphigus vulgaris is a very rare acantholytic skin disease. In most cases, oral involvement is severe, and the mouth sometimes can be the first site to exhibit lesions. Flaccid vesicles on the gums, tongue, and palate evolve rapidly into erosions and ulcerations with indistinct margins and peripheral sloughing of mucosal epithelium (Nikolsky sign). Pemphigus foliaceus, the most common form of pemphigus observed in animals, affects dogs and cats. It usually does not affect oral and other mucosal membranes.

Pemphigoid group

The pemphigoid group includes the following:

Bullous pemphigoid

Mucous membrane pemphigoid

Epidermolysis bullosa acquisita

Bullous systemic lupus erythematosus

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