A Rare Case of Aplasia Cutis Congenita of the Scalp with Dual Skin Defects in a Non-Syndromic Preterm Newborn

Aplasia Cutis Congenita (ACC) is a rare congenital condition characterized by localized or widespread absence of skin at birth. It most commonly affects the scalp and can be classified into different subtypes based on clinical presentation and associated anomalies. The exact etiology remains unclear, with genetic predisposition, vascular compromise, teratogen exposure, and maternal factors being implicated. This case is of a preterm male neonate, born at 35 weeks of gestation to a 40-year-old primigravida mother with a history of hypothyroidism and long-term thyroxine use. The mother conceived via in vitro fertilization (IVF) after undergoing extensive fertility treatments. At birth, the neonate exhibited two well-demarcated skin defects over the left posterior parietal triangle of the scalp, without involvement of deeper structures. Clinical diagnosis of non-syndromic membranous scalp ACC was made. Conservative management with saline cleansing and topical antibiotic application was adopted. The lesions healed completely by six months with residual atrophic scarring and a ‘hair collar sign’. This case highlights a rare instance of isolated ACC in a neonate with a unique maternal history of hypothyroidism, prolonged thyroxine use, and IVF-conceived pregnancy. The successful conservative management further supports its role in appropriately selected cases.

Authors

Institutions

Publication Details

Journal
Nepal Journal of Dermatology Venereology & Leprology
Published
2025-10-21
DOI
https://doi.org/10.3126/njdvl.v23i2.75646
Primary Topic
Skin and Cellular Biology Research
Type
article
Field-Weighted Citation Impact
0.00
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

A Rare Case of Aplasia Cutis Congenita of the Scalp with Dual Skin Defects in a Non-Syndromic Preterm Newborn

Aayush Bist, Sushil Dahal, Nishant Pandey, Sabin Kandel
Nepal Journal of Dermatology Venereology & Leprology
Skin and Cellular Biology Research
article

A Rare Case of Aplasia Cutis Congenita of the Scalp with Dual Skin Defects in a Non-Syndromic Preterm Newborn

Aayush Bist, Sushil Dahal, Nishant Pandey, Sabin Kandel
article en

Abstract

Aplasia Cutis Congenita (ACC) is a rare congenital condition characterized by localized or widespread absence of skin at birth. It most commonly affects the scalp and can be classified into different subtypes based on clinical presentation and associated anomalies. The exact etiology remains unclear, with genetic predisposition, vascular compromise, teratogen exposure, and maternal factors being implicated. This case is of a preterm male neonate, born at 35 weeks of gestation to a 40-year-old primigravida mother with a history of hypothyroidism and long-term thyroxine use. The mother conceived via in vitro fertilization (IVF) after undergoing extensive fertility treatments. At birth, the neonate exhibited two well-demarcated skin defects over the left posterior parietal triangle of the scalp, without involvement of deeper structures. Clinical diagnosis of non-syndromic membranous scalp ACC was made. Conservative management with saline cleansing and topical antibiotic application was adopted. The lesions healed completely by six months with residual atrophic scarring and a ‘hair collar sign’. This case highlights a rare instance of isolated ACC in a neonate with a unique maternal history of hypothyroidism, prolonged thyroxine use, and IVF-conceived pregnancy. The successful conservative management further supports its role in appropriately selected cases.

Nepal Journal of Dermatology Venereology & LeprologyVol. 23(2)
Manipal College of Medical Sciences (NP), Kathmandu University (NP)
Openalex Percentile: Top 68%
Skin and Cellular Biology Research
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.