Epidemiology of pemphigus in Macedonia: A 15‐year retrospective study (1990–2004)

V'lckova-Laskoska, Marija; Laskoski, Dimitri S.; Kamberova, Suzana; Caca‐Biljanovska, Nina; Volckova, Nadja

doi:10.1111/j.1365-4632.2006.03010.x

Cited by 46 publications

(57 citation statements)

References 32 publications

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“…However, no data on the epidemiology of pemphigus vulgaris in western Europe have been published and all of the studies are from hospital centres in the form of either cross sectional surveys or case series; these studies are unlikely to give reliable population based statistics. 3 4 6 7 10 12 [19][20][21] The incidence of bullous pemphigoid in our study is similar to that described by Garcia-Doval and colleagues 3 ; the incidence of pemphigus vulgaris is within the range of the previous papers, and the sex distribution is also similar. 9 10 12 The increasing trend in incidence of bullous pemphigoid has not been previously shown, but no study has looked at incidence of bullous pemphigoid prospectively over time.…”

Section: Discussionsupporting

confidence: 88%

Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

Langan

Smeeth

Hubbard

et al. 2008

BMJ

566

527

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Bullous pemphigoid and pemphigus vulgaris-incidence and mortality in the UK: population based cohort study Design Retrospective historical cohort study.Setting Computerised medical records from the health improvement network, a large population based UK general practice database.Participants Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls.Main outcome measures Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation.Results 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2).Conclusions Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for in incidence are not clearly understood but have implications for identifying causative factors. are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

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Section: Discussionsupporting

confidence: 88%

Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

Langan

Smeeth

Hubbard

et al. 2008

BMJ

566

527

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“…In Brazil and Tunisia, the incidence of auto-immune pemphigus is dramatically increased suggesting the role of environmental factors [7,14,15]. To our knowledge, only 4 studies on the incidence of pemphigus have been performed in southern Europe [1,5,6,16]. Only one study investigated the incidence of auto-immune pemphigus in France [7].…”

Section: Introductionmentioning

confidence: 99%

“…Little is known about the epidemiology of auto-immune pemphigus in western Europe. Most studies concern the Mediterranean Basin or Asia Minor (Bulgaria, Macedonia, Turkey, Israel, Iran, Tunisia and Greece) [1,2,4,5,6,7,8]. The crude incidence of auto-immune pemphigus in the world varies from 0.8 to 34/10 6 inhabitants [9,10,11,12,13].…”

Section: Introductionmentioning

confidence: 99%

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Incidence of Auto-Immune Pemphigus in the Midi-Pyrénées Region in 2002–2006

et al. 2009

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Introduction: Auto-immune pemphigus is an organ-specific immune disorder due to pathogenic auto-antibodies. Both genetic and environmental factors have been associated with the occurrence of auto-immune pemphigus. Little is known about the epidemiology of auto-immune pemphigus in western Europe. Objective: To evaluate the incidence of auto-immune pemphigus in south-western France (namely the Midi-Pyrénées region) in a 5-year period between 2002 and 2006. Materials and Methods: We performed a retrospective study of the annual incidence of auto-immune pemphigus diagnosed in the Midi-Pyrénées region, between January 1, 2002, and December 31, 2006. Possible cases of pemphigus were initially selected using skin direct immunofluorescence (DIF) databases from all laboratories in the Midi-Pyrénées region. Systematic validation of all cases was performed by two of the authors (N.M., M.T.). To qualify as a case of auto-immune pemphigus, patients had to fulfil the following criteria: history of clinical signs of pemphigus as assessed by a dermatologist and a positive DIF. To be included in the study, validated cases of auto-immune pemphigus had to fulfil the following criteria: the date of first positive skin DIF between January 1, 2002, and December 31, 2006, and patient living in the Midi-Pyrénées region at the time of the first positive skin DIF. Results: Between 2002 and 2006, 91 patients with positive DIF were identified. Fifty-four patients with positive DIF did not meet the validation criteria for the study. Thirty-seven cases were included in the analysis. The crude annual mean incidence of auto-immune pemphigus between 2002 and 2006 was 2.7 cases/10⁶ inhabitants/year (95% CI: 1.87–3.69). The world-population-standardized annual mean incidence of auto-immune pemphigus was 1.55 (95% CI: 0.99–2.11). Conclusion: We report a 1.55/10⁶ inhabitants/year world-population-standardized incidence of auto-immune pemphigus in the south-west of France. A nationwide epidemiological study of pemphigus should be performed in France.

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“…15 Genellikle normal görünümlü deride, bazen eritemli zemin üzerinde gelişen vezikül ve büllerin kolay rüptürü sonucu ağrılı erode alanlar meydana gelir. Bülün çevresindeki normal görünümlü deriye basınç uygulandığında epidermisin ayrıldığı gözlenir ki buna Nikolsky belirtisi denir.…”

Section: Pemfigus Vulgarisunclassified

Pemphigus

Uçmak

2011

J Clin Exp Invest

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ÖZETPemfigus deri ve mükoz membranların büllöz, sık gö-rülmeyen otoimmün kökenli bir hastalığıdır. Hastalığın patogenezinde keratinosit yüzeyindeki proteinlere karşı gelişen otoantikorların önemli rolü mevcuttur. Pemfigusun insidansı hastalığın tipine göre ülkeden ülkeye değişiklik gösterir. Pemfigus vulgaris tüm pemfigusluların yaklaşık %80'nini oluşturmaktadır ve pemfigus foliaseustan 10 kat daha fazla gözlenmektedir. Tedavide başta sistemik glukokortikoidler olmak üzere çeşitli immünsupresifler kullanılmaktadır ve son yıllarda yeni tedavi ajanları geliştirilmiştir. Bu derlemede pemfigus epidemiyolojisi, patogenezi, kliniği ve tedavisi güncel bilgiler ışığında gözden geçirilmiştir. Anahtar kelimeler: Büllöz hastalıklar, pemfigus, kortikosteroidler, tedavi ABSTRACTPemphigus is an uncommon autoimmune mucocutaneous blistering disease, mediated by antibodies directed against proteins present on the surface of keratinocytes that provide mechanical structure to the epidermis. Incidences of the various forms of pemphigus vary from country to country. Pemphigus vulgaris consists of the 80% of pemphigus cases and it occurs 10 times more than pemphigus foliaceus. There have been several recent advances in the management of pemphigus. Treatment of with systemic steroids and cytotoxic immunosuppressants can be highly effective. This article will discuss its epidemiology, pathogenesis, clinical variants and approaches to treatment. J Clin Exp Invest 2011; 2 (4): 468-473

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Epidemiology of pemphigus in Macedonia: A 15‐year retrospective study (1990–2004)

Cited by 46 publications

References 32 publications

Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

Incidence of Auto-Immune Pemphigus in the Midi-Pyrénées Region in 2002–2006

Pemphigus

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