Autoimmune subepidermal blistering diseases in Uganda: correlation of autoantibody class with age of patients

Mulyowa, Grace K.; Jaeger, Gerold; Kabakyenga, Jerome; Bröcker, Eva‐B.; Zillikens, Detlef; Schmidt, Enno

doi:10.1111/j.1365-4632.2006.02750.x

Cited by 41 publications

(43 citation statements)

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“…For consecutive subepidermal AIBD patients' sera tested on salt-split skin, EBA serum rates differed widely from one country to another: 1.3% in the UK (Logan et al, 1987), 5% in Uganda (Mulyowa et al, 2006) and New York City (USA; Zhu et al, 1990), 8% in China (Jin et al, 1993), but 20% in North Carolina (USA; Gammon et al, 1984) and 30% in Korea (Lee, 1998). Among our 196 consecutive subepidermal AIBD patients investigated by direct immunoelectron microscopy (January 2005-June 2009), 7% had EBA.…”

Section: Discussionmentioning

confidence: 97%

Black Patients of African Descent and HLA-DRB1*15:03 Frequency Overrepresented in Epidermolysis Bullosa Acquisita

Zumelzu

Roux–Villet

Loiseau

et al. 2011

Journal of Investigative Dermatology

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Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disease (AIBD). However, higher EBA incidence and predisposing genetic factor(s) involving an HLA haplotype have been suspected in some populations. This retrospective study assessed the overrepresentation of black patients with EBA, its link with HLA-DRB1*15:03, and their clinical and immunological characteristics. Between 2005 and 2009, 7/13 (54%) EBA and 6/183 (3%) other-AIBD patients seen consecutively in our department were black (P=10(-6)); moreover 7/13 (54%) black patients and 6/183 (3%) white patients had EBA (P=10(-6)). In addition, between 1983 and 2005, 12 black patients had EBA. Finally, among the 19 black EBA patients, most of them had very atypical clinical presentations, 9 were natives of sub-Saharan Africa, 1 from Reunion Island, 7 from the West Indies, and 2 were of mixed ancestry. HLA-DRB1*15:03 allelic frequencies were 50% for African patients, significantly higher than the control population (P<10(-3)), and 21% for the West Indians (nonsignificant). High EBA frequencies have already been reported in American blacks significantly associated with the HLA-DR2. In conclusion, black-skinned patients developing EBA seem to have a genetic predisposition, and EBA should be suspected systematically for every AIBD seen in this population.

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Section: Discussionmentioning

confidence: 97%

Black Patients of African Descent and HLA-DRB1*15:03 Frequency Overrepresented in Epidermolysis Bullosa Acquisita

Zumelzu

Roux–Villet

Loiseau

et al. 2011

Journal of Investigative Dermatology

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“…Mulyowa et al [15] reported that among patients with subepidermal blistering diseases, IgG reactivity correlated significantly with older age, whereas younger patients typically demonstrated IgA reactivity (P ¼ 0.024) and concluded that the age of patients with autoimmune subepidermal blistering diseases appears to influence the immunoglobulin class of autoantibodies. Mihai and Sitaru [16] summarized the immunopathological features of autoimmune bullous diseases including MMP, and the immunological and molecular tests used for their diagnosis and monitoring.…”

Section: Diagnosismentioning

confidence: 95%

Recent advances in mucous membrane pemphigoid

Daniel

Thorne

2008

Current Opinion in Ophthalmology

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“…Hastal›k s›kl›kla nükslerle seyreder. ‹nsidans› farkl› etnik populasyonlarda y›lda milyonda 2-10 olarak bildirilmifltir [1][2][3][4][5] . Ül-kemizden yap›lan bir çal›flmada, Akdeniz bölgesinde 6 y›ll›k bir süreç içerisinde 29 hastada büllöz pemfigoid tan›s› konulmufltur 6 .…”

Section: Giriflunclassified

“…Ül-kemizden yap›lan bir çal›flmada, Akdeniz bölgesinde 6 y›ll›k bir süreç içerisinde 29 hastada büllöz pemfigoid tan›s› konulmufltur 6 . Kad›n erkek oran›n›n eflit oldu¤u çal›flmalarda bulunmakla birlikte genellikle kad›nlarda erkeklerden daha fazla s›kl›kta rastlan›l›r (K: E=1,4:1-5.1:1) [1][2][3][4][5][6][7] . BP'li hastalarda otoantikorlar bazal membrandaki hemidesmozomal BP230 ve BP180 glikoproteinine karfl› geliflir 8,9 .…”

Section: Giriflunclassified

10-Year Observation of Bullous Pemphigoid Patients

Akay¹,

Bodamyali²,

Şanlı³

et al. 2010

turkderm

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ÖzetAmaç: Büllöz pemfigoid (BP) genellikle yafll› hastalarda görülen, y›ll›k mortalitesi %19-41 aras›nda de¤iflim gösterebilen genifl gergin büllerle karakterize otoimmün büllöz bir hastal›kt›r. Bu çal›flmada, BP'li olgularda etyolojik faktörler, demografik özellikler, klinik seyir ve olgular›n tedaviye yan›tlar›n›n de¤erlendirilmesi amaçlanm›flt›r. Gereç ve Yöntem: Ocak 1999-Ocak 2008 y›llar› aras›nda BP tan›s› alan 31 olguda retrospektif olarak hastal›k süresi, efllik eden semptomlar, klinik bulgular, ilaç hikayesi, tedaviye yan›t, rekürrens oranlar› ve olas› malinite taramas› için yap›lan laboratuvar tetkileri ve radyolojik incelemeler de¤erlendirildi. Bulgular: BP tan›s› alan 31 olguda kad›n erkek oran› 19/12 olup, yafl aral›¤› 59-96 aras›nda de¤iflmekteydi (ortalama 78,8±8,67). Ortalama hastal›k süresi 16,58±18,48 ay olarak saptand›. Perilezyonel derinin direk immunfloresan incelemesinde dermoepidermal bileflkede lineer IgG ve C3 depolanmas› olgular›n 24'ünde (%77,4) görüldü. Olgular›n hiç birisinde tetikleyici faktör olarak ilaç kullan›m›na ve altta yatan maliniteye rastlanmad›. Tedavide 8 olguda ilk seçene¤in oral prednizolon oldu¤u (50-60 mg/gün) ve tümün-de tedaviye tam yan›t al›nd›¤› ancak 2 olgunun tedavi devam ederken eksitus oldu¤u görüldü. Dört olgunun ise hospitalizasyon s›-ras›nda, herhangi bir tedavi bafllanamadan, nedeni bilinmeyen bir flekilde eksitus oldu¤u görüldü. Bafllang›ç tedavisi 16 olguda oral prednizolon ve azatiyopirin ile yap›l›rken, steroid dozunun azalt›larak kesildi¤i, azatiyopirin tedavisinin ise 6 ay-1 y›la tamamland›-¤› görüldü. Hastalar tedaviler kesildikten sonra ortalama 12 ay takip edilmifl ve 11 olguda (%35) nüks gözlenmifltir. En uzun remisyon prednizolon ve azatiyopirin kombinasyonunda sa¤lanm›flt›r. Sonuç: Çal›flmam›z›n sonunda BP ile malinite ve ilaç kullan›m› aras›nda bir iliflki kurulamam›flt›r. Hastal›¤›n tedaviye yan›t› iyi olup özellikle steroid ve azatiyopirin kombinasyonunun hastal›k nüksünü azaltt›¤› bulunmufltur. Ancak hastal›¤a spesifik mortalite yük-sek olup mortaliteye neden olan faktörlerin belirlenmesine ihtiyaç bulunmaktad›r. (Türkderm 2010; 44: 61-4) Anahtar Kelimeler: Büllöz pemfigoid, ileri yafl, mortalite, tedavi SummaryBackground and Design: Bullous pemphigoid (BP) is an autoimmune skin disorder characterized by large, tense blisters of the skin. It occurs mainly in the elderly. The one year mortality associated with bullous pemphigoid ranges from 19% to 41%. We aim to analyse the etiologic factors, demographic features, clinical course and response to treatment in patients with BP. Material and Method: Between January 1999 and January 2008, 31 patients with BP were evaluated retrospectively. We assessed duration of the disease, presence of associated symptoms, clinical findings, drug intake, response to treatment, recurrence rates and also extensive laboratory testing and radiologic evaluations on each patient to clarify a possible underlying malignancy. Results: In a 10-year period, 31 patients with BP, female male ratio was 19/12, ranging in a...

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Autoimmune subepidermal blistering diseases in Uganda: correlation of autoantibody class with age of patients

Abstract: The age of patients with autoimmune subepidermal blistering diseases appears to influence the immunoglobulin class of autoantibodies. The high frequency of IgA autoantibodies in Ugandan patients may be explained by the age distribution of the Ugandan population.

Cited by 41 publications

References 28 publications

Black Patients of African Descent and HLA-DRB1*15:03 Frequency Overrepresented in Epidermolysis Bullosa Acquisita

Black Patients of African Descent and HLA-DRB1*15:03 Frequency Overrepresented in Epidermolysis Bullosa Acquisita

Recent advances in mucous membrane pemphigoid

10-Year Observation of Bullous Pemphigoid Patients

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