Pemphigus Vulgaris Pemphigus refers to a group of autoantibody-mediated intraepidermal blistering diseases characterized by loss of cohesion between epidermal cells (a process termed acantholysis). Manual pressure to the skin of these patients may elicit the separation of the epidermis (Nikolsky's sign). This finding, while characteristic of pemphigus, is not specific to this group of disorders and is also seen in toxic epidermal necrolysis, Stevens-Johnson syndrome, and a few other skin diseases. Pemphigus vulgaris (PV) is a mucocutaneous blistering disease that predominates in patients 40 years. PV typically begins on mucosal surfaces; it often progresses to a mucocutaneous disease in which fragile,. | Chapter 055. Immunologically Mediated Skin Diseases Part 2 Pemphigus Vulgaris Pemphigus refers to a group of autoantibody-mediated intraepidermal blistering diseases characterized by loss of cohesion between epidermal cells a process termed acantholysis . Manual pressure to the skin of these patients may elicit the separation of the epidermis Nikolsky s sign . This finding while characteristic of pemphigus is not specific to this group of disorders and is also seen in toxic epidermal necrolysis Stevens-Johnson syndrome and a few other skin diseases. Pemphigus vulgaris PV is a mucocutaneous blistering disease that predominates in patients 40 years. PV typically begins on mucosal surfaces it often progresses to a mucocutaneous disease in which fragile flaccid blisters rupture to produce extensive denudation of the skin Fig. 55-1 . PV typically involves the mouth scalp face neck axilla groin and trunk. PV may be associated with severe skin pain some patients experience pruritus as well. Lesions usually heal without scarring except at sites complicated by secondary infection or mechanically induced dermal wounds. Postinflammatory hyperpigmentation is usually present at sites of healed lesions for some time. Figure 55-1 Pemphigus vulgaris. A. Pemphigus vulgaris demonstrating flaccid bullae that are easily ruptured resulting in multiple erosions and crusted plaques. B. Pemphigus vulgaris almost invariably involves the oral mucosa and may present with erosions involving the gingiva buccal mucosa palate posterior pharynx or the tongue. B Courtesy of Robert Swerlick MD. Biopsies of early lesions demonstrate intraepidermal vesicle formation secondary to loss of cohesion between epidermal cells . acantholytic blisters . Blister cavities contain acantholytic epidermal cells which appear as round homogeneous cells containing hyperchromatic nuclei. Basal keratinocytes remain attached to the epidermal basement membrane hence blister formation is within the suprabasal portion .
