Med Bull Sisli Etfal Hosp. 2007; 41(3): 13-18

The demographic and clinical features of autoimmune bullous dermatoses

Koray Özkan, Ilknur Kývanç Altunay, Adem Köþlü
Department of Dermatology, Þiþli Etfal, Training and Research Hospital, Istanbul, Turkey

Objective: Autoimmune bullous disorders (ABD), which occur on the genetic background and characterized with vesicles and bullae, have high morbidity and mortality rates epidemiologically. They exhibit distinctive features in terms of demographic and clinical characteristics and display diagnostic and therapeutic difficulties. Our aim to evaluate and manifest the clinical and demographic features of the inpatients with ABD, between Jan 1997 and Jan 2007;to document data and review the literature.
Methods and Materials: The medical records of the patients with ABD admitting to our dermatology department between Jan 1997 and Jan 2007 were evaluated. Demographic and clinical features including anatomic localisation of the lesions, associated disorders, complications were recorded by microsoft access database software.
Results and Conclusion: 79 patients with ABD were enrolled to the study, (mean age: 52,96 and female/male ratio was 50/29.) The most frequently seen ABD was pemphigus vulgaris (PV %49,36) and bullous pemhigoid (BP, %32,9I) and pemphigus herpetiformis and linear IgA bullous disease were the least frequent OBD with a ratio of %1,26. The patients with PV had longest duration of hospitalization with a mean duration of 34,2 days The lesions were most frequently seen on the back region of trunk (%60). Oral mucosal lesions were most frequently seen on the patients with PV.The OBD with the longest period before the diagnosis was pemphigus vegetans. Our all data were compatible with the literature.

Keywords: Autoimmune bullous dermatosis, pemphigus vulagris, bullous pemphigoid

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Otoimmun büllü dermatozlarda demografik ve klinik özellikler

Koray Özkan, Ilknur Kývanç Altunay, Adem Köþlü
Þiþli Etfal Eðitim ve Araþtýrma Hastanesi, Dermatoloji Kliniði, Ýstanbul

Amaç: Otoimmün büllü dermatozlar (OBD) genetik zeminde geliþen, vezikül ve büllerle seyreden, morbidite ve mortalitesi yüksek hastalýklardýr. Bu dermatozlar, demografik ve klinik özellikler açýsýndan farklýlýklar göstermekte, tanýsal ve tedavi zorluklarý yaratmaktadýr. Amacýmýz OBD’li hastalarda klinik, demografik özelliklerin araþtýrýlmasý ve bulgularýn belgelendirilmesidir.
Gereç ve Yöntem: Ocak 1997 -Ocak 2007 tarihleri arasýnda kliniðimizde yatarak tedavi gören OBD’li hastalarýn epikriz ve genel arþiv dosyalarý incelendi. Demografik özellikler, klinik bulgular kaydedilerek literatür gözden geçirildi.
Bulgular: Çalýþmaya toplam 79 OBD hastasý alýndý, (yaþ or¬talamasý 52.96, kadýn/erkek, 50/29).En sýk OBD Pemfigus Vulgaris (PV, %49,36), en nadir OBD. Pemfigus Herpetiformis ve Lineer IgA dermatozu (%1,26) idi. PV hastalarý orta-lama 34,2 günle en uzun yatýþ süresine sahipti. En sýk anatomik tutulum yeri gövde arka yüz lokalizasyonuydu. (% 60). Oral mukozal lezyonlar en sýk PV’ de görüldü.. Taný almadan önce ortalama yakýnma süresi en uzun olan hastalýk, 89,16 ay ile Pemfigus Vejetans idi.
Sonuçlar: Bulgularýmýz literatürle benzerlik göstermekteydi.

Anahtar Kelimeler: Otoimmün büllü dermatoz, pemfigus vulgaris, büllöz pemfigoid

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