The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels

Harman, K. E.; Seed, Paul; Gratian, M J; Bhogal, B.S.; Challacombe, Stephen; Black, Martin M.

doi:10.1046/j.1365-2133.2001.04132.x

Cited by 230 publications

(224 citation statements)

References 46 publications

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“…Skin integrity was maintained by Dsg-1, so cutaneous involvement will occur after Dsg-1 was damaged as well. 2,8,9 There were predisposition factors that trigger PV, such as drugs, ultraviolet radiation, diet of certain foods, and psychological stress. The patient in this case report had PV flared up every time she had family problems.…”

Section: Discussionmentioning

confidence: 99%

Psychological Stress and Diabetes Mellitus As Complicating Factors in Management of Pemphigus Vulgaris

Ramadian

Sarsito

2017

UIP HEATH MED

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Introduction: Pemphigus vulgaris (PV) is an autoimmune disease with vesiculobullous lesions that attack the skin and mucosa including oral mucosa. There are some factors that can trigger the recurrence of PV; one of them is psychological stress. PV is treated using immunosuppressant agents like corticosteroids or other immunosuppressant drugs. Complete remission in PV often hard to achieve because of some trigger factors that is hard to control and systemic disease like diabetes mellitus (DM).

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Section: Discussionmentioning

confidence: 99%

Psychological Stress and Diabetes Mellitus As Complicating Factors in Management of Pemphigus Vulgaris

Ramadian

Sarsito

2017

UIP HEATH MED

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“…Hastalar›n tedavi öncesi ve e¤er nüks geliflmiflse nüks esnas›n-daki hastal›k fliddetleri Harman ve ark. 13 taraf›ndan önerilen skorlama sistemine göre deri ve oral mukoza için ayr› olmak üzere derecelendirildi. Çal›flmaya al›nan hastalardan tedavi öncesinde, ayn› gün lezyonlu deri kenar›ndan doku örne¤i ve periferik venöz kan al›n-d›.…”

Section: Gereç Ve Yöntemunclassified

“…ELISA ile saptanamayan intrasellüler desmoglein antikorlar›, anti-dsg 1 negatif oldu¤u halde oluflan deri lezyonlar›n› aç›klayabilir. Hassas bir tan› yöntemi olarak kullan›lan ELISA ile desmoglein antikorlar›n›n tespitinin ayn› zamanda hastal›k fliddeti ile de iliflkili oldu¤u gösterilmifltir 3,12,13,16 . Bu çal›flmada anti-dsg-3 antikor serum dü-zeyleri oral mukoza fliddet derecelendirmesi ile iliflkili, deri fliddet derecelendirmesi ile iliflkisiz iken; anti-dsg-1 antikor serum düzeyle-ri deri fliddet derecelendirmesi ile iliflkili, oral mukoza fliddet derecelendirmesi ile iliflkisiz idi.…”

Section: Tart›flmaunclassified

“…Ancak do¤ru yorumun yap›-labilmesi için bu olgular›n uzun süreli takipleri gerekmektedir. Çal›flmam›zda daha önce yap›lm›fl çal›flmalar 3,12,13,16,30 ile uyumlu olarak ELISA ile saptanan serum desmoglein otoantikor de¤erle-rinin hastal›k fliddeti ve aktivitesi ile uyumlu oldu¤unu gözlemle-dik. D‹F incelemelerin de hastal›k aktivitesi ile paralel seyir göster-di¤ini saptad›k.…”

Section: Tart›flmaunclassified

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Pemfigusta Desmoglein Antikor Serum Düzeyleri ile Direkt İmmünofloresan Bulgularının Hastalığın Klinik Aktivitesi ile İlişkisi

Yilmaz¹,

Başkan²,

Budak³

et al. 2011

Turkderm

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ÖzetAmaç: Pemfigus deri ve mukozalarda bül oluflumuyla seyreden, otoimmün bir hastal›kt›r. Bu çal›flmada bül oluflumunda rolü olan desmoglein-1 (dsg-1) ve desmoglein-3 (dsg-3)'e karfl› oluflmufl antikorlar›n saptanmas›nda kullan›lan iki yöntem olan direkt immün-floresan (IF) inceleme ve ELISA yönteminin hastal›k aktivitesi ve remisyonla iliflkisi araflt›r›lmaktad›r. Gereç ve Yöntem: Çal›flmaya 23'ü pemfigus vulgaris, 2'si pemfigus foliaseus tan›s› alm›fl toplam 25 hasta al›nd›. Hastalar›n tedavi öncesi ve klinik remisyonun 3., 6. ve 12. aylar›ndaki anti-dsg-1 ve anti-dsg-3 serum antikor düzeyleri ELISA ile araflt›r›ld›. Efl zamanl› olarak aktif hastal›kta lezyon kenar›ndan, remisyonda ise sa¤lam kalça derisi/alt dudak mukozas›ndan direkt IF inceleme yap›ld›. Nüks halinde tetkikler tekrarland›. Bulgular: Pemfigus vulgaris hastalar›n›n tedavi öncesi 17'sinde (%73,9) anti-dsg-1 antikoru, hepsinde (%100) anti-dsg-3 antikoru pozitif saptand›. ‹ki pemfigus foliaseus olgusunda tedavi öncesi anti-dsg-1 pozitif de¤erlerde iken anti-dsg-3 negatif saptand›. Antidsg-1 antikor serum düzeyleri deri fliddet skoru ile (r: 0,577; p: 0,003), anti-dsg-3 antikor serum düzeyleri ise oral mukoza fliddet skoru ile korele idi (r: 0,539; p: 0,008). Tam remisyona giren hastalar›n 16's›nda (%84,2) tedavi öncesi direkt IF'da saptanan birikim remisyonla birlikte negatifleflti. Nüks gözlenen 9 hastan›n hepsinde nüks s›ras›nda anti-dsg-1 ve/veya anti-dsg-3 serum düzeylerinde art›fl saptand›. Dokuz olgunun 3'ünde ise klinik remisyon halinde iken nüksten 1-4 ay öncesinde serum antikor düzeylerinde yük-selme tespit edildi. Sonuç: Bu çal›flmada serum desmoglein otoantikor de¤erlerinin hastal›k fliddeti ve aktivitesi ile iliflkili olabilece¤ini saptad›k. Klinik remisyon esnas›nda desmoglein antikorlar›n›n seri ölçümleri takip ve tedavi modifikasyonunda yol gösterici olabilir. (Türkderm 2011; 45: 77-82) Anahtar Kelimeler: Pemfigus vulgaris, pemfigus foliaseus, direkt immünfloresan, desmoglein antikorlar›, hastal›k aktivitesi Summary Background and Design: Pemphigus is an autoimmune disease that results in blistering of the skin and mucous membranes. In this study, we investigated the relationship between disease activity and remission with ELISA scores and direct immunofluorescence (IF) -two methods used for the detection of antibodies against desmoglein-1 (dsg-1) and desmoglein-3 (dsg-3) that are responsible for blister formation. Material and Method: Twenty-three pemphigus vulgaris patients and two pemphigus foliaceus patients were enrolled in the study. The serum levels of anti-dsg-1 and anti-dsg-3 antibodies were measured with ELISA before therapy and at 3, 6, and 12 month of clinical remission. Concurrently, direct IF was performed on perilesional skin during active disease and on normal buttock skin/lower lip mucosa in remission. The tests were repeated if relapse has occured. Results: Anti-dsg-1 was detected in 17 (73.9%) pemphigus vulgaris patients and anti-dsg-3 in 23 (100%) pemphigus vulgaris patients. In two pemphigus foliaceus patient...

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“…More recently, specific ELISAs have become available for measurement of individual antidesmoglein antibody levels. 10,11 A number of lines of evidence indicate that these antibodies are truly pathogenic. Titres of antibodies tend to correlate with severity of disease and injection of antibodies into neonatal mice leads to acantholytic blistering.…”

Section: Pathogenesismentioning

confidence: 99%

Pemphigus: a brief review

Groves

2009

Clinical Medicine

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Pemphigus is an uncommon autoimmune blistering disease, mediated by antibodies directed against desmosomal adhesion proteins (most particularly desmogleins 1 and 3) that are responsible for maintaining integrity of the epidermis. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment. EpidemiologyRobust epidemiologic data on pemphigus incidence are scarce but a recent study from the UK suggests an incidence of 0.68 cases per 100,000 person years. 1 Incidence varies in different parts of the world, being more common in the Near and Middle East than in Western Europe and North America. Those with Jewish ancestry also seem to have a particularly high incidence. 2 All ages can be affected by pemphigus, though it is most common in middle age, and both sexes are affected equally. Several studies on the effect of smoking on pemphigus seem to suggest that, at least in some populations, it has a protective effect. 3 A rare endemic form of pemphigus foliaceus (fogo selvagem) has been identified in parts of Brazil and in North Africa. 4 The cause remains to be identified, but cases seem to cluster in rural villages around rivers and resolve when patients move away from the endemic areas. It has been suggested that the disease may be transmitted by insects living in the area.Before the advent of systemic steroid therapy pemphigus was often a lifethreatening disease. Morbidity is now more often a result of therapy than related to the underlying disorder. Disease duration varies widely, though remission rarely occurs in less than three years. Some patients require ongoing therapy for over 30 years. GeneticsThe genetics of pemphigus are complex. 5,6 Familial cases are uncommon but there are strong associations with certain genes in the MHC complex on chromosome 6, some of which vary according to the population studied. Thus, in Jewish populations DRB1*0402 is highly overexpressed whereas this is less common in Western Europeans. Conversely, the DRB1*1401 allele is highly overexpressed in European and Japanese groups. 6 Interestingly, genetic polymorphisms have recently been identified in the desmoglein 3 antibody target gene that associate with the disease and are in epistasis with the MHC associations. 7 PathogenesisThe primary pathogenic event in all forms of pemphigus is acantholysis, the separation of epidermal keratinocytes from each other. This leads to blister formation within the epidermis and is a key histological diagnostic feature of the disease (Fig 1).The immunopathology of pemphigus is increasingly well understood. 8,9 Antibodies develop against various elements of the desmosomes, particularly desmogleins 1 and 3. Desmoglein 1 is found particularly in the superficial layers of the epidermis; antibodies against this protein alone result in pemphigus foliaceus. Desmoglein 3 is more widely distributed in the lower parts of the epidermis and in mucosal epithelia. Thus, antibodies against this protein are associated with pemphigus vulgaris, typically presenting with muc...

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The severity of cutaneous and oral pemphigus is related to desmoglein 1 and 3 antibody levels

Abstract: This study suggests that the clinical phenotype of pemphigus, in particular the balance of skin and oral disease, is determined principally by the quantities of Dsg1 and 3 autoantibodies, respectively.

Cited by 230 publications

References 46 publications

Psychological Stress and Diabetes Mellitus As Complicating Factors in Management of Pemphigus Vulgaris

Psychological Stress and Diabetes Mellitus As Complicating Factors in Management of Pemphigus Vulgaris

Pemfigusta Desmoglein Antikor Serum Düzeyleri ile Direkt İmmünofloresan Bulgularının Hastalığın Klinik Aktivitesi ile İlişkisi

Pemphigus: a brief review

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