High Risk of Death in Elderly Patients With Extensive Bullous Pemphigoid

Roujeau, Jean‐Claude; Lok, C.; Bastuji‐Garin, Sylvie; Mhalla, Sami; Enginger, Véronique; Bernard, Philippe

doi:10.1001/archderm.134.4.465

Cited by 181 publications

(197 citation statements)

References 12 publications

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“…BP'nin mortalite oranı pemfigus vulgarise göre çok daha düşüktür. Mortaliteye katkıda bulunan faktörler, BP'nin daha çok yaşlı kişilerde görülmesi, kadın cinsiyet, genel durum bozukluğu, eşlik eden sistemik hastalıkların varlığı, tedavide yüksek doz kortikosteroidlerin verilmesi ile ilişki olarak bilinmektedir 21,24,26 . Avrupadan, BP'nin bir yıllık mortalite oranlarına yönelik çok çeşitli yayınlar mevcuttur.…”

Section: Introductionunclassified

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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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Section: Introductionunclassified

“…Avrupadan, BP'nin bir yıllık mortalite oranlarına yönelik çok çeşitli yayınlar mevcuttur. Bu çalışmalarda mortalite oranı %10,8-41 olarak belirlenmiştir 20,21,24,25 . Ülkemizden Akay ve arkadaşlarının yaptıkları çalışmada ise mortalite oranı %19,3 olarak hesaplanmıştır 16 .…”

Section: Introductionunclassified

Büllöz pemfigoidli hastalarda 3 yıllık gözlem: 29 olgu

Ekiz¹,

Şen²,

Rifaioğlu³

et al. 2013

TURKDERM

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“…15,16 Although previous case studies and observational studies reveal that, in addition to infection, cardiovascular events or thromboembolic diseases are important causes of death in patients with BP, the risk of stroke after the diagnosis of BP relative to the age-matched general population remains unknown. 12,17 The goal of this study is to investigate the risk of stroke in patients with BP compared with unaffected individuals of a similar age, adjusted for underlying comorbidities, using a randomly selected nationwide population-based sample.…”

mentioning

confidence: 99%

Increased Risk of Stroke in Patients With Bullous Pemphigoid

Yang

Chen

Xirasagar

et al. 2011

Stroke

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Background and Purpose-Although previous research reveals that cardiovascular events and thromboembolic diseases are important causes of death in patients with bullous pemphigoid (BP), the risk of stroke after the diagnosis of BP relative to the general population remains unknown. Using a randomly selected nationwide population-based sample, this study investigates the risk of stroke in patients with BP compared with unaffected individuals of a similar age. Methods-This study analyzes data from Taiwan's National Health Insurance Research Database. This sample included 390 patients with BP and 1950 matched subjects as a comparison group. Stratified Cox proportional hazard regressions were used to calculate the 3-year stroke risk for these 2 groups after adjusting for patient's age, sex, and comorbid medical disorders at baseline. Results-Of the 2340 patients in the sample, 312 patients (13.3%) had strokes during the 3-year follow-up period, 89(22.8% of the patients with BP) in the study group and 223 (11.4% of patients without BP) in the comparison group (PϽ0.001). The hazard ratio for stroke for patients with BP was 2.37 (95% CI, 1.78 to 3.15; PϽ0.001) times as high that for patients without BP within the 3-year follow-up period after adjusting for hypertension, diabetes, hyperlipidemia, heart failure, atrial fibrillation, and coronary heart disease. Conclusions-Patients

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“…BP may be associated with autoimmune diseases, inflammatory dermatoses, malignancies, or neurological disorders. Various neurological diseases, in particular, have been reported in association with BP, including cerebrovascular disease, dementia, multiple sclerosis, epilepsy, Parkinson's disease, Shy Drager syndrome, and amyotrophic lateral sclerosis [1][2][3].…”

Section: To the Editormentioning

confidence: 99%

Poor response to therapy for bullous pemphigoid lesions located on the hemiplegic half of the body

Yaşar¹,

Cebeci²,

Aytekin³

et al. 2016

Glob Dermatol

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To the editor,Bullous pemphigoid (BP) is an autoimmune disease prevalent in elderly patients characterized by the formation of subepidermal blisters or bullae. In BP, histopathological evidence indicates that autoantibodies are directed against two hemidesmosomal proteins, designated BP180 and BP230. Although there are theories of blister formation, the causes of the formation of autoantibodies are not fully understood. BP may be associated with autoimmune diseases, inflammatory dermatoses, malignancies, or neurological disorders. Various neurological diseases, in particular, have been reported in association with BP, including cerebrovascular disease, dementia, multiple sclerosis, epilepsy, Parkinson's disease, Shy Drager syndrome, and amyotrophic lateral sclerosis [1][2][3].A 74-year-old female patient presented with a 1.5-month history of itchy and bullous lesions containing serous fluid on different parts of her body. She had a history of diabetes mellitus, hypertension, and goitre. She had Broca's aphasia and complete right hemiplegia following a left-side cerebrovascular accident three years earlier. Dermatological examination showed flaccid bullae with bullae residues of 0.5-5cm in diameter and erosions predominately on the trunk, shoulders, arms, and legs. We performed a biopsy, in which subepidermal detachment and eosinophil predominance mixed inflammatory infiltrate were detected ( Figure 1e). In a direct immunofluorescence biopsy within perilesional skin, linear IgG and C3 deposition were found to be positive at the dermal-epidermal junction. We could not study indirect immunofluorescence BpAg1 (230 kDa) and BpAg2 (180 kDa) because we did not have the equipment to perform the test/analysis. We diagnosed her condition as BP, and treatment was started with moderate and high potency topical steroids for about two weeks. Unfortunately, she did not respond well. Because of the generalized lesions and risk of secondary infection, we engaged in systemic treatment. We started with 60 mg/day methylprednisolone and 100 mg dapsone once daily. The lesions showed resistance to dapsone treatment and she began to develop anaemia; therefore, the treatment was discontinued after 13 days. The methylprednisolone was then increased to 120 mg/ day and 150 mg/day of azathioprine was added. After increasing the systemic steroid doses, most of the lesions on the left side of the body improved; however, there was poor improvement on the right side with hemiplegia, and large blisters and bullae continued to occur ( Figures 1a, 1b, 1c and 1d).Previous studies and case reports have suggested that neurological disorders, such as dementia, Parkinson's disease, and multiple sclerosis, may be associated with BP and that the age of BP occurrence can range from 1.5 years to 60 years. Recently, an association between certain neurological conditions and BP in elderly populations has been reported [4,5]. In neurological diseases associated with BP, AG1 is a common antigen that can play a role in BP pathogenesis, with BpAG1a occurring in...

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High Risk of Death in Elderly Patients With Extensive Bullous Pemphigoid

Cited by 181 publications

References 12 publications

Büllöz pemfigoidli hastalarda 3 yıllık gözlem: 29 olgu

Büllöz pemfigoidli hastalarda 3 yıllık gözlem: 29 olgu

Increased Risk of Stroke in Patients With Bullous Pemphigoid

Poor response to therapy for bullous pemphigoid lesions located on the hemiplegic half of the body

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