PEMPHIGUS VULGARIS PEMPHIGUS VULGARIS

PEMPHIGUS VULGARIS is a skin disorder characterized by vesicles that blister on a normal or reddened background of skin. PEMPHIGUS VULGARIS je kožne reakcije značilni mehurčki, da pretisni omot na normalne ali reddened ozadja kože.

Pemphigus vulgaris is a rare (1/1000000) autoimmune intraepidermal blistering disease. Pemphigus vulgaris je redka (1 / 1000000) avtoimunske bolezni intraepidermal mehurji. This disease affects the skin, as well as the mucous membranes and is potentially life threatening. Ta bolezen prizadene kožo, kot tudi na sluznico in je lahko smrtno nevarne. Pemphigus vulgaris is found mainly in people of Ashkenazi Jewish descent and the age of onset if 60 years. Pemphigus vulgaris je ugotovljeno predvsem pri ljudeh z Aškenazi judovskega rodu in starost 60 let, če nastopa.

Causes of Pemphigus Vulgaris Vzroki za Pemphigus vulgaris

• an autoimmune blistering disease avtoimunsko bolezen, mehurji

• caused by abnormal autoimmune antibodies, IgG, which are produced against a specific component of the skin, desmoglein 3 within the epidermis of the skin ki jih povzroča nenormalne avtoimunskih protiteles IgG, ki so proizvedeni pred posebno komponento na koži, desmoglein 3 v povrhnjice na koži

• this reaction of antibody attacking a component of the skin, leads to the formation of fluid-filled vesicles or larger vesicles called bullae ta reakcija protitelo napadajo sestavni del kože, vodi k nastajanju tekočino napolnjeni mehurčki ali večjih mehurčkov imenovanih bullae

Pemphigus Vulgaris usually begins with oral lesions being evident first, then followed by skin blisters months later. Pemphigus Vulgaris običajno začne z ustnim lezije, ki očitno prva, nato sledi kožo pretisnem omotu mesecev kasneje. The oral lesions are painful erosins that dissect into the oral mucosa. Po proučitvi ustnih lezije so boleče erosins da seciram v ustni sluznici. The skin lesions, which are know as large blisters called bullae vary in size from 1-3 cm in diamteter. Na koži lezije, ki so vedeli tako veliko pretisnem omotu pozval bullae razlikujejo glede na velikost od 1-3 cm v diamteter. These may easily rupture. The bullae have a very thin outer membrane which is very fragile. Pressure can cause the bullae to alter shape, with the fluid within the bullae moving deeper into lateral epidermis. Ti lahko z lahkoto zlomiti. V bullae imajo zelo tanko zunanjo membrano, ki je zelo ranljiv. Pritisk lahko povzroči, da bullae, naj ustrezno spremeni obliko, s tekočino v bullae gibljejo globlje v bočni povrhnjice. These bullae are caused by autoimmune antibodies against intercellular skin proteins, leading to an auto-inflammatory response leading to damage of the skin and accumulation of fluid in the form of bullae and blisters. Te bullae jih povzročajo avtoimunskih protiteles proti Međućelijski kožo beljakovin, ki vodi k prilivu, auto-inflammatory odgovor vodi do poškodbe kože in kopičenje tekočine v obliki bullae in pretisnih omotih. Nikolsky's sign describes that traction pressure that is applied to the skin will lead to bullae formation. Nikolsky je znak, ki opisuje vleko tlaka, ki se nanese na kožo bo privedlo do bullae tvorbo. Bullae rupture leads to erosion formation, which can last for months before healing. Bullae pretrgu vodi do zmanjšanja tvorbe, ki lahko traja mesece, preden celjenje. Healing consists replacing the erosions with brow! Sestoji celjenje erozij nadomešča s hriba! n pigmented skin. n pigmentirana koža. Scarring however, is rare. Death can occur either from infection of the lesions or from side-effects of prolonged steroid use, which is the treatment of choice. Brazgotinjenje pa je redka. Smrt se lahko pojavi bodisi od okužba rane, ali iz stranske učinke steroidov s podaljšanim uporabo, ki je zdravljenje prve izbire. Mortality is around 5-15%. Smrtnost je okoli 5-15%.



Picture of pemphigus in a dog, showing extensive ruptured bullae on an erythematous base/inflammed skin. Slika pemphigus v psa, ki prikazuje obsežno pretrgal bullae na eritematozni baza / inflammed kožo.

Epidemiology of Pemphigus Vulgaris Epidemiology of Pemphigus vulgaris

• Often effects patients aged 40-60 and of Jeweish or Mediterranean decent Pogosto učinke pri bolnikih, starih od 40-60 in Jeweish ali sredozemskih dostojno

• Pemphigus is often associated with cancer, and other autoimmune diseases, such as myasthenia gravis Pemphigus je pogosto povezana z rakom, in drugih avtoimunskih bolezni, kot so miastenija gravis

Appearance and Body Sites Affected by Pemphigus Vulgaris Videz in telesa, ki so jih prizadele Pemphigus vulgaris

• Body sites affected: Prizadete dele telesa:

• mainly effects the MOUTH predvsem učinki v ustih

• lesions begin in the mouth, and then move to affect the rest of the body lezije začeli v usta, in nato premikate, da vplivajo na druge dele telesa

• also affects face and scalp vpliva tudi na obrazu in lasišču

• chest, armpits, and groin prsih, armpits, in sluga

• Pressure on skin leads to lesion formation and also the extension of previous lesions Pritisk na koži lezije, ki povzroča nastanek ter tudi razširitev predhodnih poškodb

• Lesions heal without scarring: however, changes in skin colour may be seen after lesions have left Lezije celijo brez brazgotinjenje: kljub temu, sprememba obarvanosti kože, se lahko obravnava po lezije so zapustile

• May be FATAL if left untreated Je lahko usodna, če levi nezdravljenih

Diagnosis of Penphigus Vulgaris Diagnoza Penphigus vulgaris

Skin biopsy's usually show intraepidermal bulla, eosinophiul infiltrate and acantholysis, which refers to the separation of epidermal cells. Rezultat biopsije kože je navadno kažejo intraepidermal Bulla, eosinophiul vtihotapiti in acantholysis, ki se nanaša na ločevanje epidermalna celic. Direct and indirect immunofluorescence, the presence of immunoglobulin G and complement C3 within the affected areas, and antibodies directed against desmoglein 3, a cellular adhesion molecule all aid in diagnosis. Neposredni in posredni imunofluorescenco, prisotnost imunoglobulina G in dopolnjujejo C3 v prizadetih območjih, in protitelesa proti desmoglein 3, celične adhezijske molekule vso pomoč pri diagnostiki. Prognosis is usually done by following antibody levels. Prognoza se običajno izvaja po naslednjih ravni protiteles.

Treatments for Pemphigus Vulgaris Obdelave za Pemphigus vulgaris

Treatment is aimed at stopping the formation of blisters. Zdravljenje je usmerjen v ustavitev oblikovanje pretisnem omotu. Prednisone is the mainstay of treatment. Prednizona je temelj zdravljenja. Adjuvant therapies, that are known to work effectively alongside prednisone in decreasing and suppressive the immune reaction that forms these blisters, includes cyclophosphamide, as well as other immunosuppressive agents. Adjuvantno zdravljenje, da je znano, da učinkovito delujejo skupaj v prednizon pada in supresivno imunski reakciji, ki tvori ta pretisni omoti, vključuje ciklofosfamid, kot tudi drugimi imunosupresivi. Intralesional steroids has recently been found to be much more effect than systemic treatment. Intralesional steroidi je bilo pred kratkim ugotovljeno, da je veliko več kot sistemski učinek zdravljenja.

• cyclophosphamide ciklofosfamid

• prednisone (steroid) is the mainstay of treatment - acts by decreasing the autoimmune response towards the skiin prednizona (steroidov) je temelj zdravljenja - deluje z manjšo avtoimunskih odgovor v smeri skiin

• predinsone at a dose of 2-3 mg/kg body weight should be started initially, and then tapered/lowered to 1-1.5 mg/kg to clear the lesions - after, steroids should always be slowly stopped due to there many side effects predinsone v odmerku 2-3 mg / kg telesne mase je treba začeti na začetku in nato stožčasti / zniža na 1-1.5 mg / kg do jasne lezije - po, steroide je treba vedno počasi nehali zaradi obstajajo številni neželeni učinki

• plasmapheresis - a procedure which removes antibodies within the body, therefore removing the IgG antibodies that are attacking the skin plazmafereza - postopek, s katerim se odstranijo protiteles v telesu, zato odstranitvijo IgG protitelesa, ki napadajo kožo

UPDATES: RECENT ADVANCES IN PEMPHIGUS VULGARIS CAUSES AND TREATMENTS Posodobitev: nedavni napredek v PEMPHIGUS vulgaris vzrokov in zdravljenja

Recently, the role of important immune cells interactions, specifically in the CD40/CD154 interaction have been implicated as a therapeutic mechanism in treating pemphigus. Nedavno je pomembno vlogo imunskih celic se medsebojnega delovanja, zlasti v CD40/CD154 interakcije so bila vpletena kot terapevtska mehanizma pri zdravljenju pemphigus. A study by Aoki-Ota et al., the was published in the Journal of Investigative Dermatology, revealed that the CD40/CD154 interaction is essential for pemphigus vulgaris, and that this is the interaction that causes the anti-Desmoglein IgG antibodies from being created. Študija, ki jo Aoki-Ota et al., Je bilo objavljeno v Journal of Investigative Dermatology, je pokazala, da je CD40/CD154 interakcij je bistvenega pomena za pemphigus vulgaris, in da se to vzajemno delovanje, ki povzroča protikonkurenčne Desmoglein IgG protitelesa, da bi ustvarili . The group further showed that blockade of this interaction holds promise for treatments, through anti-CD154 monoclonal antibodies. Skupine je nadalje pokazala, da blokado te interakcije drži obljubo za zdravljenje, z anti-CD154 monoklonskih protiteles.

Miyo Aoki-Ota, Mari Kinoshita, Takayuki Ota et al . Miyo Aoki-Ota, Mari Kinoshita, Takayuki Ota et al. Tolerance induction by the blockade of CD40/CD154 interaction in pemphigus vulgaris mouse model. Journal of Investigative Dermatology (2006) 126, 105-113. Strpnosti indukcija z blokado CD40/CD154 interakcij v pemphigus vulgaris mišjega modela. Listu Investigative Dermatology (2006) 126, 105-113.