Pemphigus Vulgaris

Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. It is classified as a type II hypersensitivity reaction, with the formation of antibodies against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. As desmosomes are attacked, the layers of skin separate and the clinical picture resemble a blister. Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn. Before the advent of modern treatments, mortality for the disease was close to 90%. Today, the mortality rate with treatment is between 5-15%.