Pemphigus vulgaris - A report of three cases and review of literature

Abstract

Pemphigus vulgaris (PV) is an autoimmune, potentially life-threatening disease causing blisters and erosions of the skin and mucous membranes associated with intraepithelial acantholysis. The underlying mechanism responsible for causing intraepithelial lesions is the binding of immunoglobulin G autoantibodies to desmoglein 3, a transmembrane glycoprotein adhesion molecule present on desmosomes. Histological features comprise intraepithelial cleft and Tzanck cells. Corticosteroids remain the mainstay of the treatment plan. In this article, we have discussed about the diagnosis of three patients suffering from PV, the treatment rendered, and the outcome of the same.

Keywords: Corticosteroids; Nikolsky's sign; Tzanck cells; vesicles.

Figure 1

Blisters were present on the bridge of nose (a), while intraorally, desquamative gingivitis was seen on the marginal gingiva in relation to 11,12, and ulcerations seen on bilateral posterior buccal mucosa (b,c and d).

Figure 2

Fresh-ruptured vesicles on the scalp (a) seen, while intraorally, irregular ulcers with ragged margins were present on the labial mucosa (b), bilateral buccal mucosa (c), and dorsum of the tongue (d) - which also exhibited a thick, diffuse, white coating.

Figure 3

Multiple painful ulcers in right buccal mucosa and soft palate (a). Stained Haematoxylin and Eosin sections [×400] revealed stratified squamous epithelium with intraepithelial blister formation, characteristic suprabasilar cleavage, and presence of acantholytic large, rounded keratinocytes with a hypertrophic nucleus and a perinuclear halo - “Tzanck cells” (c) in the split area. DIF revealed intercellular substance deposition of IgG, : characteristic “fish-net appearance” (d).