Aplasia cutis congenita in Korea: Single center experience and literature review

Pediatr Int. 2020 Jul;62(7):804-809. doi: 10.1111/ped.14192. Epub 2020 Jul 9.

Abstract

Background: 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.

Methods: 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.

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.

Conclusions: 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.

Keywords: Korea; aplasia cutis congenita; congenital; pediatricians; scalp.

Publication types

  • Review

MeSH terms

  • Administration, Topical
  • Adolescent
  • Child
  • Child, Preschool
  • Ectodermal Dysplasia / epidemiology*
  • Ectodermal Dysplasia / pathology
  • Ectodermal Dysplasia / therapy
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Minoxidil / therapeutic use
  • Pregnancy
  • Pregnancy Complications / epidemiology
  • Republic of Korea / epidemiology
  • Scalp / abnormalities
  • Scalp / pathology
  • Skin / pathology
  • Young Adult

Substances

  • Minoxidil