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Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus.Pemphigus was derived from the Greek word pemphix, meaning blister. [1] It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other.
Pemphigus is further divided in two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, several other disorders such as IgA pemphigus, IgE pemphigus, pemphigus herpetiformis, drug-induced pemphigus, Senear Usher syndrome, and endemic pemphigus foliaceus exist, and are recognized by a dermatologist from the appearance ...
If no lesions are present on examination it may be useful way of demonstrating reduced epithelial adhesion. In contrast, in Pemphigus, the epithelium tends to disintegrate rather than form a bulla. Nikolsky's sign is present in pemphigus and mucous membrane pemphigoid, but not in bullous pemphigoid.
Pemphigus erythematosus and pemphigus foliaceus both exhibit superficial blistering of the skin with minimal involvement on the mucosa (eg. mouth). This is in contrast to pemphigus vulgaris, which has antibodies against desmoglein-3 primarily. [2] Patients with pemphigus erythematosus will have positive ANA serology, a common feature of lupus. [5]
Hailey–Hailey disease (HHD), or familial benign chronic pemphigus [1]: 559 or familial benign pemphigus, [2]: 622 was originally described by the Hailey brothers (Hugh Edward and William Howard) in 1939. [3] [4] It is a genetic disorder that causes blisters to form on the skin.
Pemphigus erythematosus (Senear–Usher syndrome) Pemphigus foliaceus; Pemphigus herpetiformis (acantholytic herpetiform dermatitis, herpetiform pemphigus, mixed bullous disease, pemphigus controlled by sulfapyridine) Pemphigoid nodularis; Pemphigus vegetans; Pemphigus vegetans of Hallopeau; Pemphigus vegetans of Neumann; Pemphigus vulgaris ...
It is also associated with pemphigus vulgaris and pemphigus foliaceus. [6] [7] It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force ...
Pemphigus vegetans is a localized form of pemphigus vulgaris. [1] in which there is a localized vegetating papillomatous response. The eroded areas do not heal like usual but form papillomatous growth and vegetation. Accounts for 1-2% of pemphigus cases and is a relatively benign variant of pemphigus vulgaris. Two forms are recognized: