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Aplasia cutis congenita of the scalp accompanied by limb hypoplasia: Adamson-oliver syndrome

Title
Aplasia cutis congenita of the scalp accompanied by limb hypoplasia: Adamson-oliver syndrome
Authors
Ha Y.J.Byun J.Y.Choi Y.-W.Myung K.B.Choi H.Y.
Ewha Authors
명기범최혜영최유원변지연
SCOPUS Author ID
명기범scopusscopus; 최혜영scopus; 최유원scopus; 변지연scopus
Issue Date
2011
Journal Title
Korean Journal of Dermatology
ISSN
0494-4739JCR Link
Citation
vol. 49, no. 10, pp. 952 - 954
Indexed
SCOPUS; KCI scopus
Abstract
Aplasia cutis congenita (ACQ is an uncommon condition which is characterized by congenital, localized or widespread absence of skin or scar formation. Lesions can be multiple and may occur on any body surface, although they are mostly seen on the scalp as a solitary lesion. ACC is most often a benign isolated defect; but it can be associated with other physical anomalies or malformation syndromes. Adams-Oliver syndrome is a distinct subtype in which distal limb reduction is found in association with a solitary scalp defect. There is no single underlying cause of ACC; it may reflect a disruption of intrauterine skin development. A 25 day-old boy was referred to our clinic with a thick hemorrhagic crust, and a surrounding hairless atrophic scar on the midline over the skull vertex. He also had hypoplasia of the left 2nd to 5th toes since birth. The rest of the physical examination as well as an ultrasound examination of the brain were normal. Herein we report a rare case of ACC of the scalp accompanied by distal limb hypoplasia, Adamson-Oliver syndrome.
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의학전문대학원 > 의학과 > Journal papers
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