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Abstract
A severe case of congenital anomalies is described, with several characteristics of Beare-Stevenson syndrome, such as cutis gyrata, acanthosis nigricans, craniofacial anomalies, ear defects, enlarged umbilical stump and anogenital anomalies. He does not have craniosynostosis or clover leaf skull, which has also been described in this syndrome. This patient also shows hands and feet anomalies, absence of skin adnexa in several locations and dental anomalies, which could suggest an associated ectodermal dysplasia.
Reference
- Cutis gyratum, acanthosis nigricans and other congenital anomalies; A new syndrome.B J Dermatol. 1969; 81: 241-247
- Cutis gyratum and acanthosis nigricans associated with other anomalies; a distinctive syndrome.J Pediatrics. 1978; 92: 950-962
- Ectodermal dysplasias; a general view.J Braz Med Assoc. 1992; 38: 221-224
Article info
Publication history
Accepted:
June 10,
1992
Received:
April 2,
1992
Identification
Copyright
© 1993 The British Association of Plastic Surgeons. Published by Elsevier Inc.