Micrograph of pemphigus vulgaris with the characteristic "tombstoning". H&E stain.
Specialty
Dermatology
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. As desmosomes are attacked, the layers of skin separate and the clinical picture resembles a blister. These blisters are due to acantholysis, or breaking apart of intercellular connections through an autoantibody-mediated response.[2] 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% due to the introduction of corticosteroids as primary treatment.[3] Nevertheless, in 1998, pemphigus vulgaris was the fourth most common cause of death due to a skin disorder. It is thus still deemed "potentially fatal".
The disease mainly affects middle-aged and older adults between 50 and 60 years old. There has historically been a higher incidence in women.[4]
^Cholera M, Chainani-Wu N (June 2016). "Management of Pemphigus Vulgaris". Advances in Therapy. 33 (6): 910–958. doi:10.1007/s12325-016-0343-4. PMC 4920839. PMID 27287854.
^Beigi PK (2018). "History of Pemphigus Vulgaris". A Clinician's Guide to Pemphigus Vulgaris. Springer, Cham. p. 13. doi:10.1007/978-3-319-67759-0_3. ISBN 978-3-319-67758-3. S2CID 91065046.
^King DF, Holubar K (October 1983). "History of pemphigus". Clinics in Dermatology. 1 (2): 6–12. doi:10.1016/0738-081X(83)90019-6. PMID 6400552.
^Gupta VK, Kelbel TE, Nguyen D, Melonakos KC, Murrell DF, Xie Y, et al. (July 2011). "A globally available internet-based patient survey of pemphigus vulgaris: epidemiology and disease characteristics". Dermatologic Clinics. 29 (3): 393–404, vii–iii. doi:10.1016/j.det.2011.03.016. PMID 21605804.
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