Aplasia Cutis Congenita
- PMID: 30571024
- Bookshelf ID: NBK535403
Aplasia Cutis Congenita
Excerpt
Aplasia cutis congenita (ACC) is a rare congenital skin defect involving focal or extensive absence of the epidermis, dermis, and, occasionally, subcutaneous tissue. The exact etiology remains unclear, with proposed mechanisms including impaired prenatal skin development. Initially reported by Cordon in 1767, ACC may present as solitary or multiple lesions on any body region, with 70% to 90% localized to the vertex of the scalp. Classification includes 6 subtypes, some associated with congenital dermatologic syndromes. Most lesions heal spontaneously. However, certain locations and clinical features warrant thorough evaluation to detect underlying soft tissue anomalies that may be life-threatening.
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Sections
- Continuing Education Activity
- Introduction
- Etiology
- Epidemiology
- Pathophysiology
- Histopathology
- History and Physical
- Evaluation
- Treatment / Management
- Differential Diagnosis
- Prognosis
- Complications
- Deterrence and Patient Education
- Pearls and Other Issues
- Enhancing Healthcare Team Outcomes
- Review Questions
- References
References
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- Belkhou A, François C, Bennis Y, Duquennoy-Martinot V, Guerreschi P. [Aplasia cutis congenita: Update and management]. Ann Chir Plast Esthet. 2016 Oct;61(5):450-461. - PubMed
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- Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 1986 Apr;14(4):646-60. - PubMed
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- Lonie S, Phua Y, Burge J. Technique for Management of Aplasia Cutis Congenita of the Scalp With a Skin Allograft. J Craniofac Surg. 2016 Jun;27(4):1049-50. - PubMed
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