Pemphigus Vulgaris in Adolescence: a Case Report

Pemphigus encompasses four related diseases with autoimmune etiopathogeneses: Pemphigus vulgaris, vegetans, erythematosus, and foliaceus. Only the vulgaris and vegetans types attack the oral mucosa, and pemphigus vulgaris is the most prevalent type (Neville et al., 2009). These rare diseases involve the mucosa and skin through the disintegration of cellular adherence (acantholysis), resulting in intradermal bullous disease (Maruani et al., 2008; Neville et al.). The nasal mucosa, palpebral area, genitalia, pharynx, esophagus, and larynx can also be affected (Nishikawa et al., 1996). The annual incidence of pemphigus is around 0,1 - 0,5/100,000 individuals (Amagai et al., 1992; Neville et al.). This disease has a serious prognosis and may be fatal if it is not diagnosed and treated in the initial phase (Ahmed et al., 1980).

Pemphigus vulgaris is characterized by alterations in the intracellular links of the Malpighian stratum spinosum, where some structures become antigenic and stimulate the production of autoantibodies, mainly immunoglobulin G (IgG) (Neville et al.). The etiology of these modifications is not currently known, but the target of the autoantibodies is desmoglein-3 (Dsg3), a glycoprotein that interacts with components of the dense plaque of the desmosomes.