Lipoid proteinosis Urbach-Wiethe’s Disease

Yıl 2015, Cilt: 25 Sayı: 6, 357 - 360, 30.12.2015

Hatice Karaman Işın Soyuer İsmail Külahcı Nursen Tatlışen

Öz

A 25-year-old female patient was admitted to our outpatient clinic with postpartum hoarseness. Punch biopsy specimens obtained from the larynx and sublingual region revealed multi-folded squamous epithelium with a hyperkeratosis pattern and amorphous hyaline material aggregation. This aggregation was also remarkable around the vessels. Histochemical examination showed periodic acid-schiff-positive staining of the basal membrane with negative staining with Congo red. Physical examination revealed papule-like itchy lesions in the whole body and hoarseness. Ophthalmological examination showed bilateral blepharitis. Tooth loss and yellowish papule formation in the oral mucosa were remarkable. The patient was diagnosed with lipoid proteinosis characterized by typical vocal cord involvement and conventional histopathological and clinical findings.

Anahtar Kelimeler

Blepharitis, larynx, lipoid proteinosis

Lipoid proteinozis Urbach-Wiethe Hastalığı

Öz

Yirmi beş yaşında kadın hasta doğumdan itibaren ses kısıklığı ile polikliniğimize başvurdu. Larenks ve dilaltı bölgesinden alınan punch biyopsi örneklerinde, hiperkeratoz paternli çok katlı yassı epitel ve epitel altında amorf hiyalinize materyal birikimi izlendi. Bu birikim, özellikle damarlar etrafında da izlendi. Histokimyasal incelemede bazal membranda periyodik asit-schiff ile pozitif boyanma, kongo kırmızısı ile negatif boyanma elde edildi. Fizik muayenede tüm vücutta papül benzeri kaşıntılı lezyonlar ve ses kısıklığı saptandı. Göz muayenesinde iki taraflı blefarozis görüldü. Dişlerde kayıp ve ağız mukozasında sarı renkli papül oluşumları da dikkati çekti. Olgumuz tipik vokal kord tutulumu ve klasik histopatolojik ve klinik bulgular ile karakterize lipoid proteinozis ile tanılandı

Anahtar Kelimeler

Blefarozis, larenks, lipoid proteinozis

Kaynakça

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