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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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
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 (firstname.lastname@example.org).
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.
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