Bart sendromu: Aplazia cutis ve distrofik epidermolizis büllosa
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Dosyalar
Tarih
2016
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Otuz dokuz haftalık 3080 g olarak doğan erkek bebekte yapılan fizik muayenede her iki bacakta dizden başlayan ve ayakların da dâhil olduğu alanda cilt epitelinin olmadığı, solda daha fazla olmakla birlikte, ellerde büllöz lezyonlar görüldü. Takibinde küçük travmalarla yeni büllöz lezyonların oluştuğu izlendi. El tırnaklarında distrofik görünüm olması, Aplazia cutis ve distrofik epidermolizis büllosa birlikteliği ile Bart Sendromu tanısı konuldu. Antibiyotik ve antifungal tedavi sistemik ve lokal olarak uygulandı. Yüzeyel epitelizasyonun yeterli görüldüğü 5. haftasında aileye bakım eğitimleri verilerek taburcu edildi. Bart sendromu; cildin konjenital lokalize yokluğu (aplasia cutis), epidermolizis bülloza ve distrofik tırnak değişiklikleri ile karakterize genetik bir hastalıktır. Aplasia cutis'in epidermolizis büllosa ile birlikte bulunmasını açıklayacak birçok hipotez ortaya atılmıştır. En çok kabul gören ise prenatal dönemde ekstremitelerin sürtünmesi sonucu ortaya çıkan intrauterin bül formasyonlarına bağlı cilt kaybı durumudur
Physical examination of a 3080 g male infant born at 39, gestational week revealed extensive absence of cutaneous epithelial layer of lower extremities including feet and bullous lesions in both hands, more pronounced on the left. During follow-up period he developed new bullous lesions with minor traumas Moreover, he had nail dystrophies on both hands. He was diagnosed as Bart syndrome with the findings including aplasia cutis, epidermolysis bullosa and dystrophic nails. Systhemic and local antibacterial and antifungal treatments were started and he was discharged at fifth postnatal week when adequate superficial epithelization was achieved, he was discharged after his family received training on maintenance of his care. Bart syndrome is a rare genetic disorder characterized by conjenital skin defects (aplasia cutis), epidermolysis bullosa and dystrophic nails. Several hypothesis have been suggested to explain the co-existance of aplasia cutis and epidermolysis bullosa. Most commonly accepted hypothesis is that, cutis aplasia emerges due to the friction of affected lower extremities during intrauterne period
Physical examination of a 3080 g male infant born at 39, gestational week revealed extensive absence of cutaneous epithelial layer of lower extremities including feet and bullous lesions in both hands, more pronounced on the left. During follow-up period he developed new bullous lesions with minor traumas Moreover, he had nail dystrophies on both hands. He was diagnosed as Bart syndrome with the findings including aplasia cutis, epidermolysis bullosa and dystrophic nails. Systhemic and local antibacterial and antifungal treatments were started and he was discharged at fifth postnatal week when adequate superficial epithelization was achieved, he was discharged after his family received training on maintenance of his care. Bart syndrome is a rare genetic disorder characterized by conjenital skin defects (aplasia cutis), epidermolysis bullosa and dystrophic nails. Several hypothesis have been suggested to explain the co-existance of aplasia cutis and epidermolysis bullosa. Most commonly accepted hypothesis is that, cutis aplasia emerges due to the friction of affected lower extremities during intrauterne period
Açıklama
Anahtar Kelimeler
Bart Sendromu, Aplazia Cutis, Distrofik Epidermolizis Büllosa, Yenidoğan, Bart Syndrome, Aplasia Cutis, Dystrophic Epidermolysis Bullosa, Newborn
Kaynak
İzmir Tepecik Eğitim Hastanesi Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
26
Sayı
1
