Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita

Aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth, with 85% of cases of ACC involving the scalp vertex. The etiology of ACC is unclear and appears to be multifactorial. We present the case of a 3‐month‐old boy who presented with a diagnosis of non‐sca...

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Bibliographic Details
Published in:Pediatric dermatology 2021-03, Vol.38 (2), p.472-476
Main Authors: Davis, Matthew J., Voller, Lindsey M., Gonzalez, Santiago R., Abu‐Ghname, Amjed, Davies, Lesley W., Bedwell, Joshua R., Lee, Grace L., Hunt, Raegan D., Phung, Thuy L., Buchanan, Edward P.
Format: Article
Language:English
Subjects:
Aplasia
aplasia cutis congenita
Case reports
Congenital diseases
Dermatology
Ectodermal Dysplasia - diagnosis
Ectodermal Dysplasia - therapy
Etiology
Humans
Infant
Infant, Newborn
Male
multidisciplinary
Newborn babies
Pediatrics
Respiratory diseases
Scalp
Skin
undiagnosed disease
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Summary:Aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth, with 85% of cases of ACC involving the scalp vertex. The etiology of ACC is unclear and appears to be multifactorial. We present the case of a 3‐month‐old boy who presented with a diagnosis of non‐scalp ACC affecting approximately 80% of his total body surface area at birth. This case adds to the literature due to the patient's survival beyond the first day of life and his unique and severe distribution of defects, which led to respiratory compromise and required multidisciplinary management.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.14528