Editing Pemphigus (section)

==Types==
The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important.

*[[Pemphigus vulgaris]] (PV - [[ICD|ICD-10]] L10.0) is the most common form of the disorder and occurs when [[Antibody|antibodies]] attack [[desmoglein 3]]. Sores often originate in the mouth, making eating difficult and uncomfortable. Although PV may occur at any age, it is most common among people between 40 and 60. It is more frequent among [[Ashkenazi Jews]]. Rarely, it is associated with [[myasthenia gravis]].  [[Nail disease]] may be the only finding and has prognostic value in management.{{citation needed|date=November 2012}}<ref name=":0" />
* [[Pemphigus foliaceus]] (PF) is the least severe variety. [[Desmoglein 1]], the protein that is targeted by the autoantibodies, is enriched in the upper skin layers. PF is characterized by crusty sores that often begin on the [[scalp]], and may move to the chest, back, and face. Mouth sores do not occur. This form is also frequent among Ashkenazi Jews. It is not as painful as PV, and is often misdiagnosed as [[dermatitis]] or [[eczema]]<ref name=":0" /> PF is endemic in Brazil <ref name="pmid7805306">{{cite journal|vauthors=Sampaio SA et al | title=Brazilian pemphigus foliaceus, endemic pemphigus foliaceus, or fogo selvagem (wild fire)|journal= Dermatologic Clinics| volume=12|issue=4|pages=765–776|year=1994| doi=10.1016/S0733-8635(18)30140-2|pmid=7805306}}</ref> and Tunisia.<ref name="pmid25558949">{{cite journal|vauthors=Saleh MA | title=Pemphigus in the Arab world|journal= The Journal of Dermatology| volume=42|issue=1|pages=27–30|year=2015|pmid=25558949| doi=10.1111/1346-8138.12676| s2cid=5126848|doi-access=free}}</ref>
*[[Intraepidermal neutrophilic IgA dermatosis]] is characterized histologically by intraepidermal bullae with neutrophils, some [[eosinophils]], and acantholysis.<ref name=":0">{{cite journal | doi=10.7241/ourd.20134.158 | title=Autoimmune epidermal blistering diseases | year=2013 | last1=Abreu Velez | first1=Ana Maria | last2=Calle | first2=Juliana | last3=Howard | first3=Michael S. | journal=Our Dermatology Online | volume=4 | pages=631–646 | doi-access=free }}</ref>
* The least common and most severe type of pemphigus is [[paraneoplastic pemphigus]] (PNP). This disorder is a complication of [[cancer]], usually [[lymphoma]] or [[Castleman's disease]]. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the [[esophagus]]. In this variety of pemphigus, the disease process often involves the lungs, causing [[bronchiolitis obliterans]] (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population. Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.
*Endemic pemphigus foliaceus includes fogo selvagem, the new variant of endemic pemphigus foliaceus in El Bagre, Colombia, and the Tunisian endemic pemphigus in North Africa.<ref>{{cite journal | doi=10.1067/s0190-9622(03)00851-x | title=A unique form of endemic pemphigus in northern Colombia | year=2003 | last1=Abrèu-Velez | first1=Ana María | last2=Hashimoto | first2=Takashi | last3=Bollag | first3=Wendy B. | last4=Tobón Arroyave | first4=Sergio | last5=Abrèu-Velez | first5=Clara Eugenia | last6=Londoño | first6=Martha Luz | last7=Montoya | first7=Fernando | last8=Beutner | first8=Ernst H. | journal=Journal of the American Academy of Dermatology | volume=49 | issue=4 | pages=599–608 | pmid=14512903 }}</ref>

[[Hailey-Hailey disease]], also called familial benign pemphigus, is an inherited skin disease, not an autoimmune disease, so it is not considered part of the pemphigus group of diseases.<ref>{{Cite web |url=http://www.haileyhailey.com/WhatIsHHD.htm |title=Hailey Hailey Disease Society |access-date=2008-03-04 |archive-url=https://web.archive.org/web/20121014124508/http://haileyhailey.com/WhatIsHHD.htm |archive-date=2012-10-14 |url-status=dead }}</ref>