Bullous Diseases in Malaysia: Epidemiology and Natural History

Adam, B. A.

doi:10.1111/j.1365-4362.1992.tb03519.x

Cited by 84 publications

(85 citation statements)

References 6 publications

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“…A study from Israel including 155 patients reported a female-to-male ratio of 1.5:1 [12]. However, in our study the female-to-male ratio was approximately 1:1 for both PV and PF, which is similar to previous studies from Malaysia and Finland [13,14]. …”

Section: Discussionsupporting

confidence: 40%

Long-Term Prognosis of Pemphigus in Korea: Retrospective Analysis of 199 Patients

Kim¹,

Kim

Kim³

2011

Dermatology

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Background: Pemphigus is the most common and severe autoimmune bullous disease in Korea. Objective: The aim of this clinical study was to evaluate the clinical features, treatment modalities and long-term prognosis of pemphigus in Korea. Methods: We conducted a retrospective analysis of 199 patients diagnosed with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) between 1993 and 2008. Complete and partial remissions were evaluated according to the definitions proposed by the International Pemphigus Committee. Results: The mean age of onset was 46.1 years, and the female-to-male ratio was 1.1:1.0 (women: 102, men: 97). The ratio of PV to PF was 1.1:1.0 (PV: 104, PF: 95). Complete/partial remission rate for PV was 77% at 5 years and 94% at 10 years after initial diagnosis. The corresponding rate for PF was 87% at 5 years and 98% at 10 years after initial diagnosis. There was no difference in time to remission between mild cases (treated with prednisolone, Pd, alone) and severe cases (treated with Pd ± adjuvant therapy). Sixteen patients, who were recalcitrant to conventional therapy, received rituximab; all of these achieved overall remission without adverse effects. Eight (8%) PV patients and 5 (5%) PF patients died during the 16-year follow-up period. Conclusions: Most patients reached remission within 10 years of treatment. Pd + adjuvant therapy did not yield a better prognosis than Pd alone. All patients who received rituximab experienced remarkable clinical improvement. Rituximab is an effective and safe drug for patients who are resistant to conventional therapy.

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Section: Discussionsupporting

confidence: 40%

Long-Term Prognosis of Pemphigus in Korea: Retrospective Analysis of 199 Patients

Kim¹,

Kim

Kim³

2011

Dermatology

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“…[2][3][4] In the United Kingdom, a regional study estimated an incidence of 1.4 per 100 000 person years. 5 Wide variation in mortality is reported, with one year mortality varying between 6% in the United States and 41% in France.…”

Section: Introductionmentioning

confidence: 99%

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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“…İnsidansı yaşla birlikte artmakta ve farklı etnik popülasyonlarda değişmektedir [1][2][3] . Dünyanın farklı bölgelerinden BP ile ilgili çok sayıda klinikoepidemiyolojik çalışma bildirilmiştir [4][5][6][7][8][9][10][11][12][13][14] . Ülkemizden de BP ile ilgili 3 farklı epidemiyolojik çalışma bulunmaktadır [15][16][17] .…”

Section: Introductionunclassified

Büllöz pemfigoidli hastalarda 3 yıllık gözlem: 29 olgu

Ekiz¹,

Şen²,

Rifaioğlu³

et al. 2013

TURKDERM

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ÖzetAmaç: Büllöz pemfigoid (BP) kronik, otoimmun subepidemal büllöz bir hastalıktır. Çalışmamızdaki amaç, son 3 yılda kliniğimizde BP tanısı konulan olguların klinik ve epidemiyolojik özelliklerini belirlemek ve sonuçlarımızı ülkemizdeki ve dünyanın diğer yerlerindeki sonuçlarla karşılaştırmaktır. GereçveYöntem:Ocak 2009 ve Ocak 2013 tarihleri arasında Dermatoloji polikliniğimize başvuran, klinik ve histopatolojik olarak BP tanısı alan hastaların dosyaları retrospektif olarak değerlendirildi. Hastaların yaş, cinsiyet, oral mukoza tutulumu, kaşıntının varlığı, hastalık süresi, tetikleyici faktör bulunup bulunmaması, deri lezyonlarının özellikleri, biyopsi örneklerinin direk immünofloresan (DIF) inceleme sonuçları, tedavide kullanılan ilaçlar, nüks ve eksitus oranları ve saptanabilen ölüm nedenleri gibi klinik ve demografik verileri kaydedildi. Bulgular:Çalışmaya alınan 29 hastanın %48,3'ü kadın, %51,7'i erkekti. Yaş ortalamaları 70,17 yıl iken, ortalama hastalık suresi 15,41 aydı. Oral mukoza tutulumu beş, kaşıntı 24 olguda saptandı. Tetikleyici faktör olarak üç olguda ilaç, bir olguda da malinite belirlendi. DIF inceleme sonuçlarında dermoepidermal bileşkede lineer IgG ve C3 depolanması 12 olguda, C3 depolanması 10 olguda mevcuttu. Hastalara başlangıç tedavisi olarak 11 hastaya topikal kortikosteroid, altı hastaya tetrasiklin and nikotinamid kom-binasyonu, 10 hastaya sistemik kortikosteroid tedavisi verildi. Yedi hastada verilen ilk tedaviden sonra bir yıl içinde nüks meydana geldi. Hastane içi mortalite iki hastada görüldü. Sonuç: Çalışmamızda son 3 yılda kliniğimizde BP tanısı konulan hastalarının klinik ve epidemiyolojik verileri sunulmuştur. İlimiz farklı etnik gruplardan oluşan heterojen bir popülasyon olduğu için, çalışmamızın ülkemizdeki BP ile ilgili epidemiyolojik verilerin oluşturulmasında katkıda bulunacağına inanıyoruz. Ancak bu konuda yapılmış prospektif, daha uzun süreli ve çok merkezli çalışmalara ihtiyaç duyulmaktadır. Sum maryBackgroundandDesign:Bullous pemphigoid (BP) is a chronic, autoimmune, subepidermal blistering skin disease. The aim of this study was to determine the clinico-epidemiological characteristics of patients diagnosed with BP in our clinic and to compare the results with those in other studies in our country and in the world. Materials and Methods:We retrospectively evaluated medical records of patients with BP diagnosed by clinical and histopathological examinations in our clinic from January 2009 to January 2013. We evaluated data on demographic characteristics, age, sex, existence of oral mucosal lesions, association of pruritus, duration of the disease, presence of triggering factors, features of the skin lesions, results of direct immunofluorescence (DIF) investigations, drug intake, recurrence and mortality rates and causes of de-tected mortality in all patients. Results:Of the 29 patients included in the study, %48.3 were female, %51.7 were male. The average age was 70.17 years and the mean duration of the disease was 15.41 months. We detected oral mucosal involvement in...

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Bullous Diseases in Malaysia: Epidemiology and Natural History

Cited by 84 publications

References 6 publications

Long-Term Prognosis of Pemphigus in Korea: Retrospective Analysis of 199 Patients

Long-Term Prognosis of Pemphigus in Korea: Retrospective Analysis of 199 Patients

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

Büllöz pemfigoidli hastalarda 3 yıllık gözlem: 29 olgu

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