Bullous aplasia cutis congenita: A report of two cases and brief review of the selected literature

Vojnosanitetski Pregled

卷:76

Matić Milan¹  Gajinov Zorica¹  Stašuk Nataša²  Matić Aleksandra³  Prćić Sonja³  Jablanović Slađana⁴ 

[1] Clinical Center of Vojvodina, Clinic of Dermatovenereology Diseases, Novi Sad + Faculty of Medicine, Novi Sad;

[2] Clinical Center of Vojvodina, Clinic of Ginecology and Obstestrics, Novi Sad;

[3] Institute for Child and Youth Health Care of Vojvodina, Pediatric Clinic, Novi Sad + Faculty of Medicine, Novi Sad;

[4] Institute for Child and Youth Health Care of Vojvodina, Pediatric Clinic, Novi Sad ;

关键词: congenital abnormalities;    ectodermal dysplasia;    scalp;    hypertrichosis;    hemangioma;   

DOI  :  10.2298/VSP170201076P

来源: DOAJ

【 摘 要 】

Introduction. Aplasia cutis congenita (ACC) is a rare condition characterized by the focal absence of skin, and sometimes other underlying structures at birth. It may occur as an isolated defect or associated with other anomalies and defects. Bullous ACC (BACC) is a clinical subtype of the condition with few cases reported in the literature. It presents as a bullous lesion at birth which gradually transforms into an atrophic scar covered by a thin epithelial membrane. It is considered as cutaneous sign of possible neural tube dysraphism. Some cases present with a dark hair around the lesion (the hair collar sign), which can be even more indicative of neural tube defect. However, cases of BACC reported till today are inconclusive regarding this connection. Case report. We report a two cases of BACC of the scalp, in one patient associated with hair collar sign without neural tube defects and the other with hemangioma and we give a brief review of the selected literature. Conclusion. Bullous or membranous aplasia cutis congenita is benign condition, but may represent as a cutaneous marker of occult neural tube defect. Recognising the condition is important in order to rule out associated anomalies.

【 授权许可】

Unknown