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Pemphigus Vulgaris | Open Access Journals
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Journal of Immunobiology

ISSN: 2476-1966

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Pemphigus Vulgaris

A 36-year-old woman had a burning gum sensation that increased with the consumption of spicy foods. Intra-oral examination revealed a severe generalized erythema with detachment of the surface layer of the gum. An extra-oral examination of the skin of the hand revealed multiple bubbles with a positive Nikolsky 1 sign and Asboe-Hansen sign or Nikolsky 2 sign. Perilesional biopsy samples of the gum and skin showed acanthholytic blisters and intercellular edema in the lower epithelium. Direct immunofluorescence has demonstrated the presence of IgG in the intercellular regions of the epithelium. The desmoglein 3 ELISA test was positive for anti-desmoglein  antibodies in serum. Based on the above investigations, the diagnosis arrived was Pemphigus vulgaris. The treatment instituted was the topical application of 0.1% triamcinolone acetonide to the oral lesions for 4 weeks and systemic prednisolone 80 mg for 2 weeks. Systemic prednisolone was reduced in stages for an additional 4 weeks; oral and skin lesions regressed after 6 weeks. The gingival and skin lesions healed completely without leaving scars at the one-year follow-up visit. Pemphigus vulgaris is an autoimmune intraepithelial and blistering disease of the skin and mucous membranes.1 The incidence rate of pemphigus is 1 to 16 cases per year per million people.2 Pemphigus vulgaris is associated with the binding of IgG autoantibodies to desmogelin 3, a transmembrane adhesion molecule of the glycoprotein present on desmosomes causing loss of intercellular adhesion and separation of cells called acantholysis in the lower layers of stratum spinosum, which leads to eventually the formation of suprabasilar bubbles.3 The differential diagnosis of Pemphigus vulgaris includes aphthous stomatitis, lichenplanus, oral candidiasis and pemphigoid which can be identified by clinical results, immunofluorescence tests, histopathological examination and acid test Periodic Schiff using smear samples for candidate hyphae. Treatment consists of systemic and topical steroids with constant follow-up visits to prevent recurrence of the disease.

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