APLASIA CUTIS CONGENITA ON SCALP WITH CALVARIAL BONE DEFECT, DOUBLE URETER AND DOUBLE RENAL PELVIS.
- Author:
Sae Hwi KI
;
Chang Eun JEUNG
;
Eun Ryoung KIM
- Publication Type:Original Article
- MeSH:
Bandages;
Child;
Ectodermal Dysplasia*;
Extremities;
Female;
Humans;
Infant;
Karyotype;
Kidney;
Kidney Pelvis*;
Pelvis;
Scalp*;
Skin;
Skull;
Ureter*;
Wills;
Wounds and Injuries
- From:Journal of the Korean Society of Plastic and Reconstructive Surgeons
1997;24(3):503-507
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Aplasia cutis congenita represents a congenital absence of all skin layers, and it may occasionally extend through the bone and dura of the skull. Since the first report was described in the extremity by Cordon 1767, and Campbell 1826, approximately over 500 cases have been reported. About eighty five percents of all cases are found in the scalp, with 15 to 30 percents involving the skull as well. Fifteen percents of all cases involve nonscalp locations and are often bilateral symmetrical. It has been relatively rarely reported disorder abroad as well as domestically. It has several clinical subtypes classified by the location and pattern of skin absence, the presence of associated malformation and the mode of inheritance with unknown cause. We had a new born female infant with this disorder, who presented with a full thickness skin defect on scalp and skull defect. No skin defect were reported in other family members, including a first child born several years previously. Chromosomal analysis revealed as normal female karyotype, but she had double pelvis and double ureter of both kidney The legions healed for five weeks by conservative treatment as moist wound dressing and systemic antibiotic administrations. As a relatively uncommon skin anomaly with congenital anomaly, one case of aplasia cutis congenita involving scalp and skull is reported with the review of reference.
