Pemphigus vulgaris: environmental factors. Occupational, behavioral, medical, and qualitative food frequency questionnaire

ABSTRACT:Pemphigus is a group of potentially life-threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examinations, is essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected by both normal human skin and monkey esophagus or by enzyme-linked immunosorbent assay (ELISA). Before the introduction of corticosteroids, pemphigus vulgaris was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using systemic corticosteroids, with azathioprine, dapsone, methotrexate, cyclophosphamide, and gold as adjuvants or alternatives, but mycophenolate mofetil and intravenous immunoglobulins also appear promising.

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“…These findings suggested that this may point to the contribution of estrogens in the disease process (Brenner et al, 2001).…”

Section: Other Factorsmentioning

confidence: 84%

Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

Scully

Challacombe

2002

Critical Reviews in Oral Biology & Medicine

139

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“…Bu verilerin aksine Valikhani ve Bastuni araştırmalarında gebelik sayısında bir artış saptamamış, ancak pemfiguslu hastalarda oral kontraseptif kullanımı-nın daha sık olduğunu bildirmişlerdir. 6,10,11 Bizim hastalarımızın çok sayı-da gebelik geçirmiş olmaları çoğunun Doğu ve Güneydoğu Anadolu bölgelerinden geliyor olmalarına ve o bölgelerde Türkiye geneline göre gebelik oranı ve doğum hızının yüksek olmasına bağlı olabilir. 12 Pemfigusun bazı otoimmün hastalıklarda olduğu gibi infertilite ile ilişki-si olduğu da ileri sürülmüştür.…”

Section: Discussionunclassified

Pemfigusta Risk Faktörleri

Demirci¹,

Mansur²,

Aydıngöz³

2011

TURKDERM

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ÖzetAmaç: Pemfigusun ortaya çıkmasında ve hastalığın seyrinde bazı dış faktörlerin etkili olduğu üzerinde durulmaktadır. Bu araştırma-da pemfigus tanısı ile takip ettiğimiz hastalarda hastalığın etyolojisinde rol oynayabilecek faktörlerin araştırılması amaçlanmıştır. Gereç ve yöntem: Kliniğimizde 1998-2004 yılları arasında histopatolojik ve direkt immunofloresan incelemeler ile pemfigus tanı-sı konan 15'i erkek, 27'si kadın toplam 42 hasta, demografik özellikler, meslek grubu, eğitim düzeyi, hastalığın ortaya çıkmadan önceki süreçte geçirilmiş stresli yaşam olayları, gebelik sayısı, diyet, sigara ve alkol alışkanlığı gibi etkenler açısından sorgulanarak benzer sosyoekonomik düzeye sahip, aynı yaş ve cinsiyetteki bir kontrol grubuyla karşılaştırıldı. Bulgular: Hayvancılık ve tarım ile uğraşmanın, gebelik sayısının fazla oluşunun, sigara içmemenin ve psikolojik stresin pemfiguslu hastalarda kontrol grubuna göre istatistiksel olarak anlamlı oranda yüksek bulunduğu saptandı. Sum maryBackground and Design: There have been reports suggesting the involvement of environmental factors in the disease process of pemphigus. In this study, we aimed to find out the risk factors which could play role in the etiopathogenesis in our pemphigus patients. Material and method: A total of 42 patients (15 male and 27 female) who were diagnosed as pemphigus with histopathological and direct immunoflurosence examinations in our clinic between the years 1998-2004, were interviewed for assessment of regarding with the subjects of the demographic properties, occupational groups, educational level, the number of pregnancies, stressfull life events, diet habits, smoking and alcohol consumption before the onset of the disease and the results were compared to 42 age and gender-matched controls with similar socioeconomic circumstances. Results: Working in agriculture and livestock, multi-parity, absence of smoking and stressfull life events were found to be statistically significant in pemphigus patients than in controls. Conclusion: Working in agriculture and livestock, multi-parity, absence of smoking and stressfull life events were assumed to play role in the etiopathogenesis and course of pemphigus. (Turk derm 2011; 45: 140-5)

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“…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.…”

Section: Epidemiologymentioning

confidence: 99%

“…Paget's disease is an intra-epidermal carcinoma seen in the nipple and areola area (mammary Paget's) or anogenital and axillary skin (extramammary Paget's). 3 Paget's disease of the breast presents as unilateral, sharply demarcated nipple eczema or erosion, often with bleeding or nipple discharge, in association with underlying intraductal adenocarcinoma of the breast or non-invasive carcinoma. Extramammary Paget's disease is characterised by an itchy, moist, well-defined erythematous plaque, typically in the anogenital area and most common in elderly women.…”

mentioning

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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Pemphigus vulgaris: environmental factors. Occupational, behavioral, medical, and qualitative food frequency questionnaire

Cited by 129 publications

References 49 publications

Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

Pemphigus Vulgaris: Update on Etiopathogenesis, Oral Manifestations, and Management

Pemfigusta Risk Faktörleri

Pemphigus: a brief review

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