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Öğe Bullous disorders in Konya: A study of 93 cases(Deri Zuhrevi Hastaliklar Dernegi, 2013) Daye, Munise; Mevlitoglu, Inci; Esener, SarenurBackground and Design: Bullous diseases are a group of disorders caused by specific antibodies against tissue adhesion molecules in the skin and mucosa or by genetic inheritance. There are limited detailed epidemiological studies about bullous disorders in Turkey. In this study, we evaluated the demographic, clinical and immunopathological findings as well as course of the disease and follow-up properties in patients with bullous disorders in the 'Province of Konya. Materials and Methods: Ninety-three patients, who were followed up between 2006 and 2012, were included in our study: Since most of the patients were in pemphigus and bullous pemphigoid groups, we investigated data on those patients in more detail. Results: 53.8% of patients were female and 46.2% were male. The mean age was 52.9. 37.6% of patients had pemphigus vulgaris, - 34.4% bullous pemphigoid, - 15%hereditary epidermolysis bullosa, - 4.3% dermatitis herpetiformis, - 4.3% pemphigus foliaceus, - 2.2% pemphigus vegetans, - 1.1% Hailey-Hailey disease, and 1.1% of patients had cicatricial pemphigoid. The mean age of onset of pemhigus vulgaris was 45.9 years. Pemhigus vulgaris, which was most frequently observed in females, started at oral mucosa and the time of diagnosis was longer than in patients in the other geographic regions. The mean age of onset of bullous pemphigoid was 70 years and it was mostly observed in females. In 12.5 of cases, the initial manifestations occured the oral mucosa. None of the patients had a concomitant autoimmune disorder. Twenty-five cases were treated with systemic corticosteroids, 41 cases with systemic corticosteroids and adjuvant drugs; 11 patients received only adjuvant therapy, and 16 patients were administered only local steroids. Conclusion: We report the demographical, clinical and immunopathological, findings as well as course of the disease and follow-up properties in patients with bullous disorders in the Province of Konya.Öğe Konya ilinde büllöz hastalıklar: 93 olgu üzerine bir çalışma(2013) Daye, Munise; Mevlitoğlu, İnci; Esener, SarenurAmaç: Büllöz hastalıklar; deri ve mukozaları tutan, doku adezyon moleküllerine karşı spesifik antikorlarla gelişen ya da genetik kalıtımın neden olduğu bir grup hastalıktır. Ülkemizde bu hastalıkları inceleyen detaylı epidemiyolojik çalışma sınırlıdır. Bu amaçla Orta Anadoluda Konya yöresi ve civarında yaşayan büllöz hastalığı olan olguların demografik, klinik, immünopatolojik, tedavi ve takiplerindeki özellikleri sunmaktayız. Gereç ve Yöntem: Çalışmamızda 2006-2012 yılları arasında büllöz hastalık tanısıyla takip edilmiş olan 93 olgunun verileri retrospektif değerlendirildi. Olgularımızın çoğunluğu pemfigus ve büllöz pemfigoid grubunda olduğu için onlara ait veriler daha detaylı irdelendi. Bulgular: Çalışmaya dahil edilen 93 olgunun 50'si (%53,8) kadın, 43'ü (%46,2) erkekti. Yaş ortalaması 52,9 yıldı. Olgulardan %37,6'sı pemfigus vulgaris, %34,4'ü büllöz pemfigoid, %15'i herediter epidermolizis bülloza, %4,3'ü dermatitis herpetiformis, %4,3'ü pemfigus foliaseus, %2,2'i pemfigus vegetans, %1,1'i Hailey-Hailey hastalığı %1,1'i skatrisyel pemfigoid tanısı almıştı. Pemfigus vulgarisin ortaya çıkış yaş ortalaması 45,9 yıldı ve kadınlarda daha fazla görüldü. Pemfigus vulgarisli olgularda hastalık oral mukozadan başladı ve diğer coğrafik bölgelere göre; oral mukoza tutulumu ile başlayan pemfigus vulgarisli olgularda tanı alma süresi daha uzundu. Büllöz pemfigoidin ortaya çıkış yaş ortalaması 70 yıldı ve kadınlarda daha fazla görüldü. Olguların %12,5'inde hastalık oral mukozadan başladı. Hiçbir olguda eşlik eden otoimmün hastalık saptanmadı. Doksan üç olgunun 25'ine sadece sistemik steroid, 41'ine sistemik steroid ve adjuvan, 11'ine sadece adjuvan, 16'sına sadece lokal tedaviler verildi. Sonuç: Konya ili ve civarında yaşayan büllöz hastalığı olan olguların demografik, klinik, immünopatolojik, tedavi ve takiplerindeki özellikler sunulmaktadır. (Türkderm 2013; 47: 200-4)Öğe Lipoid proteinosis (Urbach-Wiethe disease): A case report(Turkish Soc Dermatology Venerology, 2014) Daye, Munise; Dogan, Seda; Mevlitoglu, Inci; Esener, Sarenur; Toy, HaticeLipoid proteinosis, which is known as Urbach-Wiethe disease, was first described in 1929 as lipoidosis cutis et mucosae by Urbach and Wiethe. It is a rare autosomal recessive inherited genodermatosis. There are PAS-positive hyaline material deposits in the skin, mucosa and visceral organs. A 23-year-old man, who was suffering from swelling of the lower lip and a fissure on the inner side of the lip, presented to our outpatient clinic. On dermatological examination, there were lower lip edema and infiltration, as well as macroglossia. There were bilateral yellowish papules along his eyelashes. For his ocular findings; it was learned that he had been treated for trichiasis at his childhood. There were multiple atrophic scars on his face, elbows, axilla, knees, and the skin overlying his spinous processes. He had no hoarseness. There was no consanguinity between his mother and father. Physical examination was normal. Three punch biopsies were carried out from his tongue, lips and elbow with the preliminary diagnoses of lipoid proteinosis, Melkersson-Rosenthal syndrome, focal epithelial hyperplasia, amyloidosis, granulomatous cheihtis, atrofoderma, and anetoderma. Deposition of hyaline PAS-positive materials was observed on his histopathological examination. According to the clinical and histopathological findings, the patient was diagnosed as having lipoid proteinosis. Otorhinolaryngologists, ophthalmologists, dermatologists, and neurologists should keep this rare condition in mind in the differential diagnosis.
