Pemphigus Vulgaris Skin and Oral cavity ?

More than half of the patients also develop flaccid blisters and widespread cutaneous erosions. Pemphigus Vulgaris is therefore divided into two sub groups.

1) The mucosal-dominant type with mucosal erosions but minimal skin involvement and

2)The mucocutaneous type with extensive skin blisters and erosion in addition to mucosal involvement.

Mucous membrane lesions usually present as painful erosions. Intact blisters are rare, probably because they are fragile and break easily. Although scattered and extensive erosions may be seen anywhere in the oral cavity, the most common sites are the buccal and palatine mucosa. The erosions are of different sizes with and irregular and ill-defined border, which, when, extensive or painful may result in decreased oral intake of food or liquids. The diagnosis of Pemphigus Vulgaris tends to be delayed in patients presenting with only oral , involvement as compared to patients with skin lesions.

The lesions may extend out onto the vermilion lip and lead to thick , fissured hemorrhagic crusts. Involvement of the throught produces hoarseness and difficulty in swallowing. The esophagus also may be involved and sloughing of its entire lining in the form of a cast has been reported.

The conjuncivae , nasal mucosa , vagina, labia, penis and anus can develop lesions as well.Cytology of vaginal cells may be misread as a malignancy when vaginal lesions are present.

The primary skin lesions of Pemphigus Vulgaris are flaccid, thin walled, easily ruptured blisters. They can appear anywhere on the skin surface and arise on either normal-appearing skin or erythematous bases. The fluid within the bullae is initially clear but may become hemorrhagic, turbid or even seropurulent. The blisters are fragile and soon rupture to form painful erosions that ooze and bleed easily.These erosions offten attain a large size and can become generalized.The erosions soon become partially covered with crusts that have little or no tendency to heal.Those lesions that do heal often leave hyperpigmented patches with no scaring.

Because of an absence of cohesion within the epidermis , its upper layers easily move laterally with slight pressure or rubbing in patients with active disease (Nikolsky sign).

The lack of cohesion of the skin may also be demonstrated with the “bulla-spread phenomenon “ – gentle pressure on an intact bulla forces the fluid to spread under the skin away from the site of pressure (Asboe-Hansen sign, also referred to as the “indirect Niklosky” or Niklosky-II sign).