Yang Min-Young, Ha Dae-Lyong, Kim Hoon-Soo, Ko Hyun-Chang, Kim Byung-Soo, Kim Moon-Bum
Department of Dermatology, Pusan National University Hospital, Busan, Korea.
Biomedical Research Institute, Pusan National University Hospital, Busan, Korea.
Pediatr Int. 2020 Jul;62(7):804-809. doi: 10.1111/ped.14192. Epub 2020 Jul 9.
Aplasia cutis congenita (ACC) is a rare congenital malformation characterized by a localized absence of skin. which most commonly affects the scalp. We performed the present study to elucidate the basic clinical data regarding ACC in Korea, including demographics, clinical features, radiological and therapeutic results.
Fifty-nine patients (70 lesions) with ACC (35 from our department and 24 from a Koreamed database search) were enrolled. We assessed demographics, family and obstetrical histories, clinical features (multiplicity, subtype, size, shape, hair collar sign, location, and Frieden's classification), and radiologic and therapeutic results.
The mean age of patients was 2.62 years, with a male-to-female ratio of 1.03. A minority of patients had a family history (three patients), birth trauma (one patient), maternal drug use (two patients), or human immunodeficiency virus infection (one patient) during pregnancy, and fetus papyraceus of placental infarcts (two patients). Six patients (6/59, 10.17%) had multiple lesions. Scarring was the most common manifestation (39/70, 55.71%). The scalp was the most commonly affected site (50 cases, 71.43%). Thirty-nine patients (66.10%) met Frieden's type I classification (scalp ACC without multiple anomalies). Radiological investigations were performed in 30 patients (30/59, 50.85%) with abnormal findings in eight patients. Twenty-five patients (42.37%) were managed conservatively, and 17 patients (28.81%) were treated with local wound care.
This is the first and largest study assessing the basic clinical data of ACC in Korea. The results of the present study could be useful for pediatricians and dermatologists who routinely manage ACC.
先天性皮肤发育不全（ACC）是一种罕见的先天性畸形，其特征为局部皮肤缺失，最常累及头皮。我们开展本研究以阐明韩国ACC的基础临床数据，包括人口统计学、临床特征、影像学及治疗结果。
纳入59例ACC患者（70处病灶）（35例来自本科室，24例通过韩国医学数据库检索获得）。我们评估了人口统计学、家族史和产科病史、临床特征（病灶数量、亚型、大小、形状、发领征、位置及弗里登分类）以及影像学和治疗结果。
患者的平均年龄为2.62岁，男女比例为1.03。少数患者有家族史（3例）、出生时创伤（1例）、母亲孕期用药（2例）或人类免疫缺陷病毒感染（1例），以及胎盘梗死的纸样胎儿（2例）。6例患者（6/59，10.17%）有多处病灶。瘢痕形成是最常见的表现（39/70，55.71%）。头皮是最常受累部位（50例，71.43%）。39例患者（66.10%）符合弗里登I型分类（无多发畸形的头皮ACC）。30例患者（30/59，50.85%）进行了影像学检查，其中8例有异常发现。25例患者（42.37%）接受保守治疗，17例患者（28.81%）接受局部伤口护理。
这是评估韩国ACC基础临床数据的首个也是规模最大的研究。本研究结果可能对经常诊治ACC的儿科医生和皮肤科医生有用。
