Editing Pemphigus (section)

{{Short description|Blistering autoimmune diseases}}
{{About|a skin blistering disease|another similar-looking blistering skin disease|Pemphigoid|the genus of true bugs|Pemphigus (bug)}}

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| caption         = Pemphigus on the upper body
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[[File:Permphigus.jpg|thumb|Pemphigus, from 1886 medical book]]
[[Image:Pemphigus immunofluorescence.jpg|thumb|200px|Microscopic image of direct [[immunofluorescence]] using an anti-[[Immunoglobulin G|IgG]] antibody. The tissue is skin from a patient with '''Pemphigus vulgaris'''. Note the intercellular IgG deposits in the [[Epidermis (skin)|epidermis]] and the early intraepidermal [[vesicle (dermatology)|vesicle]] caused by acantholysis.]]

'''Pemphigus''' ({{IPAc-en|ˈ|p|ɛ|m|f|ɪ|g|ə|s}} or {{IPAc-en|p|ɛ|m|ˈ|f|aɪ|g|ə|s}}) is a rare group of [[blister]]ing [[autoimmune disease]]s that affect the [[skin]] and [[mucous membrane]]s.<ref name="yeh">
{{cite journal|vauthors=Yeh SW, Ahmed B, Sami N, Ahmed AR | title = Blistering disorders: diagnosis and treatment| journal = [[Dermatologic Therapy]]| volume = 16| issue = 3| pages = 214–23| year = 2003| pmid = 14510878| doi = 10.1046/j.1529-8019.2003.01631.x| doi-access = free}}</ref> The name is derived from the Greek root ''pemphix'', meaning "blister".<ref>{{Cite web|url=https://www.merriam-webster.com/dictionary/pemphigus|title=Definition of PEMPHIGUS|website=www.merriam-webster.com|language=en|access-date=2017-03-11}}</ref>

In pemphigus, [[autoantibody|autoantibodies]] form against [[desmoglein]], which forms the "glue" that attaches adjacent [[Epidermis (skin)|epidermal]] cells via attachment points called [[desmosomes]]. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes detached, a phenomenon called [[acantholysis]]. This causes [[blister]]s that slough off and turn into [[Ulcer (dermatology)|sores]]. In some cases, these blisters can cover a large area of the skin.<ref>[http://pemphigus.org/index.php?option=com_content&view=article&id=364&Itemid=100073/ International Pemphigus & Pemphigoid Foundation: What is Pemphigus?]</ref>

Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa.  In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters.<ref>{{cite journal|last1=Beutner|first1= EH|last2= Jordon|first2 =RE|title= Demonstration of skin antibodies in sera of pemphigus vulgaris patients by indirect immunofluorescent staining|journal = Proceedings of the Society for Experimental Biology and Medicine|date=November 1964|volume = 117|issue= 2|pages = 505–510|pmid=14233481|doi=10.3181/00379727-117-29622|s2cid= 9443044}}</ref><ref>{{cite web |url=http://www.dermfnd.org/press01/beutner.html |title=Dermatology Foundation: BEUTNER, JORDAN SHARE 2000 DERMATOLOGY FOUNDATION DISCOVERY AWARD |access-date=2009-01-31 |archive-url=https://web.archive.org/web/20080518082952/http://www.dermfnd.org/press01/beutner.html |archive-date=2008-05-18 |url-status=dead }}</ref> In 1971, an article investigating the autoimmune nature of this disease was published.<ref>{{cite journal|doi=10.1111/1523-1747.ep12293273|authorlink1=Robert E. Jordon|authorlink4=Ernst H. Beutner|first1=Robert E.|last1=Jordon|first2 = W. Mitchell|last2 = Sams Jr.|first3= Gustavo|last3=Diaz|first4=Ernst H.|last4=Beutner|title=Negative Complement Immunofluorescence in Pemphigus|journal=Journal of Investigative Dermatology|year=1971|volume= 57|issue=6|pages=407–410|pmid=4108416|doi-access=free}}</ref><ref>{{cite journal|last=Serratos|first=BD|author2=Rashid, RM|title=Nail disease in pemphigus vulgaris|journal=Dermatology Online Journal|date=Jul 15, 2009|volume=15|issue=7|pages=2|doi=10.5070/D34X05D6VH |pmid=19903430|url=http://escholarship.org/uc/item/4x05d6vh|url-access=subscription}}</ref>