Pemphigus
1209518
224907563
2008-07-10T23:07:27Z
Davidruben
101637
rmv uncited, ungramatical and non-country-specified addition
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = |
Caption = |
DiseasesDB = |
ICD10 = {{ICD10|L|10||l|10}} |
ICD9 = {{ICD9|694.4}} |
ICDO = |
OMIM = 169600 |
OMIM_mult = Benign Chronic ~<br/>{{OMIM2|169610}} ~ Vulgaris, Familial |
MedlinePlus = 000882 |
eMedicineSubj = derm |
eMedicineTopic = 317 |
eMedicine_mult = <br/>{{eMedicine2|derm|318}} ~ foliaceus<br/>{{eMedicine2|derm|314}} Drug-induced ~<br/>{{eMedicine2|derm|543}} ~ herpetiformis<br/>{{eMedicine2|derm|315}} ~ IgA<br/>{{eMedicine2|derm|319}} ~ Vulgaris<br/>{{eMedicine2|derm|535}} ~Paraneoplastic<br/>{{eMedicine2|derm|150}} Benign ~<br/> ([[Hailey Hailey Disease (Familial Benign Pemphigus)|Hailey-Hailey Disease]]) |
MeshID = D010392 |
}}
[[Image:Pemphigus immunofluorescence.jpg|thumb|200px|Microscopic image of direct [[immunofluorescence]] using an anti-[[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)]] caused by acantholysis.]]
'''Pemphigus''' (from the Greek ''pemphix'': bubble or blister), is an [[autoimmune]] disease of the [[skin]] and [[mucous membrane|mucous membranes]] that causes [[Blister|vesicles]] (blisters), [[bulla|bullae]] and raw sores. It may be triggered by medications, such as [[penicillamine]].
In pemphigus, [[autoantibody|autoantibodies]] form against [[desmoglein]]. Desmoglein 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 "unglued", a phenomenon called '''acantholysis'''. This causes [[Blister|blisters]] that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin. <ref>International Pemphigus & Pemphigoid Foundation: What is Pemphigus? http://www.pemphigus.org/content/view/16/30/lang,en/</ref>
==Types==
There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.
* The most common form of the disorder is '''pemphigus vulgaris''' (PV - [[ICD|ICD-10]] L10.0). It occurs when [[Antibody|antibodies]] attack ''[[Desmoglein 3]]''. Sores often originate in the [[mouth]], making eating difficult and uncomfortable. Although pemphigus vulgaris may occur at any age, it is most common among people between the ages of 40 and 60. It is more frequent among persons of [[Jew|Jewish]] <ref>[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=169610 Omim - Pemphigus Vulgaris, Familial<!-- Bot generated title -->]</ref>, Middle Eastern, or Mediterranean descent. Rarely, it is associated with [[myasthenia gravis]].
* '''Pemphigus Foliaceus''' is the least severe of the three varieties. ''[[Desmoglein 1]]'', the protein that is destroyed by the autoantibody, is only found in the top dry layer of the skin. Pemphigus foliaceus (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. It is not as painful as pemphigus vulgaris, and is often mis-diagnosed as [[dermatitis]] or [[eczema]].
* The least common and most severe type of pemphigus is '''[[paraneoplastic]] pemphigus''' (PNP). This disorder is a complication of [[cancer]], usually [[lymphoma]] and [[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]] (BOOP). Complete removal and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.
Note that [[Haley-Haley disease]], also called '''familial benign pemphigus''', is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases. <ref>Hailey Hailey Disease Society http://www.haileyhailey.com/WhatIsHHD.htm</ref>
==Diagnosis==
Pemphigus is recognized by a [[dermatologist]] from the appareance and distribution of the skin lesions. Definitive diagnosis requires examination of a skin [[biopsy]] by a [[dermatopathologist]]. The skin biopsy is taken from the edge of a blister, prepared for [[histopathology]] and examined with a [[microscope]]. The pathologist looks for an intraepidermal [[Vesicle (dermatology)|vesicle]] caused by the breaking apart of [[Epidermis (skin)|epidermal]] cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the "floor" of the blister. This bottom layer of cells is said to have a "tombstone appearance".
Definitive diagnosis also requires the demonstration of anti-desmoglein autoantibodies by [[Immunofluorescence|direct immunofluorescence]] on the skin biopsy. These antibodies appear as [[IgG]] deposits along the [[desmosomes]] between epidermal cells, a pattern reminiscent of chicken wire. Anti-desmoglein antibodies can also be detected in a blood sample using the [[ELISA]] technique. A high titre of cANCA (cellular Anti [[Neutrophil]] [[Cytoplasmic]] [[Antibody]]) is an important feature of the disease.
==Treatment==
If not treated, pemphigus can be fatal due to overwhelming [[infection]] of the sores. The most common treatment is the administration of oral steroids, especially [[prednisone]]. Intravenous [[gamma globulin]] (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus. Mild cases sometimes respond to the application of topical steroids. Recently, [[Rituximab]], an anti-[[CD20]] antibody, was found to improve otherwise untreatable severe cases of Pemphigus vulgaris. <ref name="pmid17065638">{{cite journal |author=Ahmed AR, Spigelman Z, Cavacini LA, Posner MR |title=Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin |journal=N. Engl. J. Med. |volume=355 |issue=17 |pages=1772–9 |year=2006 |pmid=17065638 |doi=10.1056/NEJMoa062930}}</ref><ref name="pmid17687130">{{cite journal |author=Joly P, Mouquet H, Roujeau JC, ''et al'' |title=A single cycle of rituximab for the treatment of severe pemphigus |journal=N. Engl. J. Med. |volume=357 |issue=6 |pages=545–52 |year=2007 |pmid=17687130 |doi=10.1056/NEJMoa067752}}</ref>
All of these drugs may cause severe side effects, so the patient should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.
If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune suppressant drugs is sometimes used in an attempt to halt the rapid progression of [[bronchiolitis obliterans]], including [[solumedrol]], [[ciclosporin]], [[azathioprine]] and [[thalidomide]]. [[Plasmapheresis]] may also be useful.
If skin lesions do become infected, [[antibiotic]] may be prescribed. In addition, [[talcum powder]] is helpful to prevent oozing sores from adhering to bedsheets and clothes.
==Pemphigus in domestic animals==
[[Image:Canine pemphigus foliaceus 3.jpg|thumb|Pemphigus foliaceus skin eruption on the ventral abdomen of a dog]]
Pemphigus foliaceus has been recognized in pet dogs, cats and horses and is the most common autoimmune skin disease diagnosed in veterinary medicine. Pemphigus foliaceus in animals produces clusters of small vesicles that quickly evolve into pustules. Pustules may rupture, forming erosions or become crusted. Left untreated, pemphigus foliaceus in animals is life-threatening leading to loss of condition and secondary infection.
Pemphigus vulgaris is a very rare disorder described in pet dogs and cats. Paraneoplastic pemphigus has been identified in pet dogs.
==Footnotes==
{{reflist|2}}
== External links ==
*[http://www.pemphigus.org/ International Pemphigus Foundation]
*[http://www.emedicine.com/DERM/topic319.htm Pemphigus vulgaris], article from eMedicine.
*[http://www.emedicine.com/derm/topic318.htm Pemphigus foliaceus], article from eMedicine.
*[http://www.emedicine.com/derm/topic535.htm Paraneoplastic pemphigus], article from eMedicine.
*[http://www.alotoflife.com/2007/10/i-am-living-with-lymphoma-non-hogkins.html A Lot of Life! Paraneoplastic Pemphigus Survivor]
*[http://www.skincosmos.com/pemphigus-vulgaris/ Pemphigus Vulgaris]
*[http://www.blisters.org.au/ www.blisters.org.au]
* {{DermAtlas|65817101}}
* http://tray.dermatology.uiowa.edu/PemVul01.htm
{{Vesiculobullous disease}}
{{Diseases of the skin and subcutaneous tissue}}
[[Category:Dermatology]]
[[Category:Autoimmune diseases]]
[[Category:Oral pathology]]
[[Category:Ashkenazi Jews topics]]
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