Pediatrics

Aplasia Cutis Congenita (ACC): A Guide for Parents and Caregivers

At a Glance

Aplasia cutis congenita (ACC) is a rare condition where a newborn is missing a patch of skin, usually on the scalp. While it looks alarming, most cases are isolated and heal excellently over time with simple, conservative daily wound care at home.

Finding a raw or open wound on your newborn’s head can be a deeply shocking and frightening experience. In the quiet, high-stakes environment of a delivery room, seeing a medical team appear surprised or uncertain only adds to that panic.

Aplasia cutis congenita (ACC) is a rare condition where a baby is born missing a small area of skin, most commonly on the scalp. While it looks alarming, the prognosis for most infants is excellent, and the condition usually heals effectively with simple, conservative wound care.

This resource guide is designed to empower parents and caregivers with clear, evidence-based information about ACC. It will help you understand the diagnosis, recognize warning signs, and collaborate effectively with your child’s medical team.

Navigating This Guide

What is Aplasia Cutis Congenita?

Learn what Aplasia Cutis Congenita (ACC) is, why your newborn has missing skin on their scalp, and why the prognosis is excellent with simple wound care.

Causes and Diagnosis of ACC

Learn about the causes and diagnosis of Aplasia Cutis Congenita (ACC). Understand vascular disruption, genetic factors, dermoscopy, and the hair collar sign.

Types and Classifications of ACC

Learn about the types of Aplasia Cutis Congenita (ACC). Understand the Frieden classification system, from isolated scalp defects to Adams-Oliver syndrome.

Warning Signs and Potential Risks

Learn the crucial warning signs of aplasia cutis congenita (ACC). Understand the hair collar sign, how to spot infections, and when your baby needs an MRI.

Treating ACC: Wound Care and Surgery

Learn about treatment options for Aplasia Cutis Congenita (ACC). Discover why conservative wound care is usually preferred and when surgery becomes necessary.

The Long-Term Outlook for Your Child

Learn about the long-term outlook for a child with Aplasia Cutis Congenita (ACC). Understand scar healing, permanent hair loss, and future cosmetic options.

Common questions in this guide

What is aplasia cutis congenita (ACC)?

Aplasia cutis congenita is a rare condition where a baby is born missing a small patch of skin, usually on the scalp. While it can look like an alarming open wound, it is usually an isolated issue that heals very well with proper care.

How is aplasia cutis congenita treated?

For most infants, ACC is treated with simple, conservative wound care using specific daily dressings. The area usually heals on its own over time, though severe cases might require a surgical evaluation.

What is the hair collar sign?

The hair collar sign is a ring of darker, longer hair around the missing skin patch. Doctors look for this because it can be a warning sign indicating underlying bone or brain involvement, which may require an MRI to investigate further.

Will my baby's aplasia cutis congenita leave a bald spot?

Yes, once the ACC lesion heals, it typically leaves a scar that will not grow hair, resulting in permanent alopecia (baldness) in that specific spot. As your child grows, your doctor can discuss future cosmetic options.

What are the signs of infection in an ACC wound?

Warning signs of infection include increased redness, swelling, warmth, or unusual drainage from the wound. If you notice any of these signs, or suspect a hemorrhage, you should contact your pediatric care team immediately.

Curated prompts to bring to your next appointment.

1.Does my child's Aplasia cutis congenita appear to be isolated, or does it fall into a specific Frieden classification group?
2.Are there any indicators, such as the hair collar sign, that suggest we should schedule an MRI to check for underlying bone or brain involvement?
3.What is the recommended wound care plan for my baby's specific lesion, and what type of dressings should we be using?
4.What specific warning signs of infection or hemorrhage should we look for, and who do we call if an emergency happens?

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This guide provides educational information about aplasia cutis congenita for parents and caregivers. Always consult your pediatrician or pediatric dermatologist for specific wound care instructions and medical advice for your baby.

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