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Pediatrics

What Causes Aplasia Cutis Congenita During Pregnancy?

At a Glance

Aplasia cutis congenita (ACC) is almost always a random developmental event and is not caused by typical pregnancy activities. It occurs when fetal blood flow to the skin is interrupted, which can be linked to vanishing twin syndrome, certain viral infections, or genetics.

If your baby was recently diagnosed with aplasia cutis congenita (ACC), it is completely normal to ask yourself, “Did I do something to cause this?” The short and most important answer is no. ACC—a rare condition where a baby is born missing a small patch of skin, most commonly on the scalp [1]—is almost always a chance occurrence [2]. It is not caused by what you ate, how much you exercised, a stressful event at work, or typical pregnancy activities.

To help ease your mind, it helps to understand the actual biological mechanisms that doctors believe cause this condition. ACC happens when the skin either fails to form completely or is disrupted while the baby is developing in the womb [2]. Here is what the medical research tells us about why this happens.

Interruptions in Blood Flow (Vascular Disruption)

One of the most widely accepted theories for why ACC occurs is a sudden, temporary drop in blood flow to a specific area of the baby’s developing skin [3][4]. If a tiny patch of tissue does not receive a steady supply of oxygen and nutrients for a brief moment, it can stop growing [5]. This is considered a random developmental glitch, much like a hiccup during the complex process of forming a baby.

Vanishing Twin Syndrome

Sometimes, a pregnancy begins with twins, but one twin stops developing very early on and is absorbed into the womb. This is known as “vanishing twin syndrome” or fetus papyraceus [6]. Often, a mother never even knows she was originally carrying twins. The loss of this twin can cause rapid blood pressure changes or vascular disruptions in the surviving baby, which is strongly linked to a specific pattern of ACC known as Type V [3][4]. Unlike the more common scalp ACC, this type usually presents on the baby’s trunk or limbs [6]. It is important to know that aside from the skin patch, the surviving baby is typically perfectly healthy.

Exposure to Specific Medications

The vast majority of standard medications, including prenatal vitamins and over-the-counter pain relievers, do not cause ACC. However, there is a known link between ACC and methimazole, a prescription drug used to treat an overactive thyroid (hyperthyroidism) [7][8]. If a mother takes methimazole during the first trimester, it can sometimes interfere with the baby’s skin development. Because of this, doctors often switch pregnant patients to alternative thyroid medications, like propylthiouracil, early in pregnancy to reduce this risk [9].

Viral Infections During Pregnancy

In very rare circumstances, contracting specific viral infections while pregnant can affect the baby’s skin. The most notable example is varicella (the virus that causes chickenpox and shingles). A varicella infection during pregnancy can leave scars or defects on the baby’s skin that present as ACC when they are born [10]. Other viruses, such as Hepatitis B, have also been associated with the condition in isolated cases [11].

Genetics and Underlying Syndromes

While the vast majority of ACC cases are isolated events meaning the baby is otherwise completely healthy, some instances are tied to a baby’s unique genetic code [12]. Certain genetic variations or syndromes, such as Adams-Oliver syndrome or Trisomy 13, include ACC as one of their symptoms [13][14].

Please do not panic if you see these syndromes mentioned online. Syndromic ACC almost always presents alongside other major, obvious medical issues (such as heart, limb, or neurological differences) that your pediatrician would likely identify immediately [15]. If your baby only has a missing patch of skin, it is highly likely to be an isolated, chance occurrence.

Looking Forward: What Does This Mean for My Baby?

Knowing why this happened does not answer your most pressing question: what happens next? The good news is that many smaller ACC lesions, especially those limited just to the skin, heal wonderfully on their own with conservative, gentle wound care [1][16]. For larger patches or those that involve deeper tissues, your care team may discuss surgical options to help close the area safely [17]. You will likely work with a multidisciplinary team—which may include a pediatrician, a pediatric dermatologist, or a plastic surgeon—to create a daily care plan that keeps your baby comfortable, prevents infection, and supports healing [18].

Common questions in this guide

Did I do something during pregnancy to cause my baby's aplasia cutis congenita?
No, you did not cause your baby's condition. Aplasia cutis congenita is almost always a random developmental event. It is not caused by your diet, stress levels, exercise routine, or other typical pregnancy activities.
How does vanishing twin syndrome cause aplasia cutis congenita?
If one twin stops developing early in pregnancy, it can cause rapid blood pressure changes for the surviving baby. This sudden shift can temporarily interrupt blood flow to the developing skin, leading to a specific type of missing skin on the baby's trunk or limbs.
Can medications taken during pregnancy cause aplasia cutis congenita?
Most standard medications and prenatal vitamins do not cause this condition. However, methimazole, a prescription medication used to treat an overactive thyroid, has been linked to the condition when taken during the first trimester.
Is aplasia cutis congenita a sign of a deeper genetic syndrome?
Most babies born with aplasia cutis congenita are perfectly healthy aside from the missing patch of skin. While the condition can occasionally be part of genetic conditions like Adams-Oliver syndrome, those syndromes almost always present with other major, obvious medical issues.
How is a missing patch of skin treated in a newborn?
Many small patches of missing skin heal very well on their own with daily, gentle wound care to prevent infection. If the patch is larger or involves deeper tissues beneath the skin, a pediatric dermatologist or plastic surgeon may recommend surgical options.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Given the location and appearance of my baby's ACC, do you suspect an isolated case or should we consult a geneticist?
  2. 2.What is the specific daily wound care routine we should follow to prevent infection and promote healing?
  3. 3.Do we need a referral to a pediatric dermatologist or a plastic surgeon for long-term monitoring?
  4. 4.Are there any deeper imaging tests, like an ultrasound of the scalp, needed to ensure there are no underlying bone or tissue issues?
  5. 5.What signs of infection or complications should I watch for when caring for the skin patch at home?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (18)
  1. 1

    Aplasia cutis congenita of the scalp: Histopathologic features and clinicopathologic correlation in a case series.

    Gassenmaier M, Bösmüller H, Metzler G

    Journal of cutaneous pathology 2020; (47(5)):439-445 doi:10.1111/cup.13644.

    PMID: 31904134
  2. 2

    Classification of aplasia cutis congenita: a 25-year review of cases presenting to a tertiary paediatric dermatology department.

    Sathishkumar D, Ogboli M, Moss C

    Clinical and experimental dermatology 2020; (45(8)):994-1002 doi:10.1111/ced.14331.

    PMID: 32501579
  3. 3

    Extensive Type V Truncal Aplasia Cutis Congenita in a Surviving Twin With Fetus Papyraceus: A Case Report.

    Alharbi AG, Nagshabandi KN, Almusa HA, et al.

    Case reports in pediatrics 2026; (2026()):4089919 doi:10.1155/crpe/4089919.

    PMID: 41635524
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    Aplasia Cutis Congenita Associated with Fetus Papyraceus.

    Pieretti ML, Alcalá R, Boggio P, et al.

    Pediatric dermatology 2015; (32(6)):858-61 doi:10.1111/pde.12651.

    PMID: 26212801
  5. 5

    Aplasia Cutis Congenita Associated With Aplasia of the Superficial Temporal Artery.

    Choi MS, Choi JH, Ki SH, Jun YH

    The Journal of craniofacial surgery 2016; (27(4)):1065-7 doi:10.1097/SCS.0000000000002678.

    PMID: 27213745
  6. 6

    Frieden's Group-V Aplasia Cutis Congenita with in utero Scarring in a Dichorionic Twin: A Case Report and Comprehensive Literature Review.

    Paraparambil Vellamgot A, Pattu Valappil R, Tomerak AH, et al.

    Case reports in dermatology 2025; (17(1)):1-8 doi:10.1159/000541676.

    PMID: 39713060
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    Management of Hyperthyroidism during the Preconception Phase, Pregnancy, and the Postpartum Period.

    Sarkar S, Bischoff LA

    Seminars in reproductive medicine 2016; (34(6)):317-322 doi:10.1055/s-0036-1593489.

    PMID: 27741549
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    Gastroschisis Following Treatment with High-Dose Methimazole in Pregnancy: A Case Report.

    Purnamasari D, Setyowati S, Yunir E, et al.

    Drug safety - case reports 2019; (6(1)):5 doi:10.1007/s40800-019-0099-2.

    PMID: 31123878
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    Comparison of the safety between propylthiouracil and methimazole with hyperthyroidism in pregnancy: A systematic review and meta-analysis.

    Liu Y, Li Q, Xu Y, et al.

    PloS one 2023; (18(5)):e0286097 doi:10.1371/journal.pone.0286097.

    PMID: 37205692
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    Aplasia Cutis Congenita as a Sole Manifestation of Congenital Varicella Syndrome.

    Leung AKC, Leong KF, Lam JM

    Case reports in pediatrics 2020; (2020()):6147250 doi:10.1155/2020/6147250.

    PMID: 32774973
  11. 11

    Type VII Aplasia Cutis Congenita in Neonates Related to Maternal HBV Infection? Case Report and Literature Review.

    Li G, Zhang A, Yang S, et al.

    Clinical, cosmetic and investigational dermatology 2023; (16()):499-504 doi:10.2147/CCID.S396071.

    PMID: 36855651
  12. 12

    Aplasia Cutis Congenita: A Case Report.

    Ugowe OJ, Balogun SA, Adejuyigbe EA

    West African journal of medicine 2021; (38(4)):391-394.

    PMID: 33904163
  13. 13

    Case Report: A novel DLL4 variant in a neonate with Adams-Oliver syndrome.

    Huang Y, Wang J, Zeng L, et al.

    Frontiers in pediatrics 2025; (13()):1532561 doi:10.3389/fped.2025.1532561.

    PMID: 40098638
  14. 14

    Aplasia Cutis Congenita of the Scalp with Bone Defect and Exposed Sagittal Sinus in Trisomy 13 Newborn - a Case Report.

    AlMatrifi FR, Al-Shammari AA, Al Nefily RM, et al.

    Medical archives (Sarajevo, Bosnia and Herzegovina) 2023; (77(4)):319-322 doi:10.5455/medarh.2023.77.319-322.

    PMID: 37876568
  15. 15

    A Case Series of Aplasia Cutis Congenita and Its Management.

    Khatija Begum M, Vijayashree J, Bathina A, Gullipalli P

    Cureus 2025; (17(3)):e80135 doi:10.7759/cureus.80135.

    PMID: 40190843
  16. 16

    An Extensive Case of Aplasia Cutis Congenita.

    Rose MD

    Cureus 2024; (16(6)):e63215 doi:10.7759/cureus.63215.

    PMID: 39070430
  17. 17

    Nonsyndromic aplasia cutis congenita: a case report.

    Chokoeva AA, Tchernev G, Patterson JW, et al.

    Journal of biological regulators and homeostatic agents 2015; (29(1 Suppl)):129-31.

    PMID: 26016981
  18. 18

    Large defects in aplasia cutis congenita treated by large-sized thin split-thickness skin grafting: long-term follow-up of 18 patients.

    Liu Y, Qiu L, Fu Y, et al.

    International journal of dermatology 2015; (54(6)):710-4 doi:10.1111/ijd.12773.

    PMID: 26010404

This page is for informational purposes only and does not replace professional medical advice. Always consult your pediatrician or dermatologist about your baby's specific condition and care plan.

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