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Dermatology

Bullous Aplasia Cutis as a Presenting Sign of Encephalocele

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JAMA Dermatology

Published Online: September 20, 2023

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Article Information and Disclosures

Jenna Borok, MD, MAS¹;

Annette M. Wagner, MD^1,2;

Anthony J. Mancini, MD^1,2

Author Affiliations

¹Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois
²Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois

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A 2-week-old male infant born at full-term after an uncomplicated pregnancy and labor presented with a congenital cystic scalp nodule. Physical examination revealed a 12 × 11-mm bullous plaque with a red-blue hue on the right vertex scalp surrounded by darker and longer hairs (Figure). A diagnosis of bullous aplasia cutis congenita (ACC) with hair collar sign was made, and magnetic resonance imaging ruled out a central nervous system tract and intracranial extension. Excision at age 6 years revealed skin devoid of hair follicles and adnexal structures with leptomeningeal-like vessels, macrophages, and cerebral cortex, findings that are consistent with ACC with encephalocele.

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Article Information

Corresponding Author: Jenna Borok, MD, MAS, Ann & Robert H. Lurie Children’s Hospital of Chicago, 225 E Chicago Ave, PO Box 107, Chicago, IL 60611-2605 (jenna.borok@gmail.com).

Published Online: September 20, 2023. doi:10.1001/jamadermatol.2023.2708

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s mother for granting permission to publish this information.

References

Colon-Fontanez F , Fallon Friedlander S , Newbury R , Eichenfield LF . Bullous aplasia cutis congenita. J Am Acad Dermatol. 2003;48(5 suppl):S95-S98.Google Scholar

Drolet B , Prendiville J , Golden J , Enjolras O , Esterly NB . ‘Membranous aplasia cutis’ with hair collars: congenital absence of skin or neuroectodermal defect? Arch Dermatol. 1995;131(12):1427-1431. doi:10.1001/archderm.1995.01690240091015 PubMed Google Scholar Crossref

Browning JC . Aplasia cutis congenita: approach to evaluation and management. Dermatologic Therapy. 2013;26(6):439-444.Google Scholar Crossref

Frieden IJ . Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 1986;14(4):646-660. doi:10.1016/S0190-9622(86)70082-0 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.