PHACES syndrome presenting as hemangiomas, sternal clefting and congenital ulcerations on the helices

The Journal of Dermatology
Cicek DurusoyErkan Alpsoy

Abstract

Sternal malformation/vascular dysplasia association is a very rare condition comprised of midline defects and hemangiomas of the face and anterior trunk, that can be found as part of the PHACES phenotypic spectrum (posterior fossa malformations, hemangiomas, arterial anomalies, coarctations of the aorta, cardiac defects and eye abnormalities, sternal clefting). Herein, we describe a 6-month-old boy with sternal cleft, extensive segmental hemangiomas, and a depigmented scar on the tip of the xyphoid process, corresponding to a sternal malformation/vascular dysplasia association. He also had bilateral cutaneous ulcerations on the helices. Our case report indicates that ulceration of a hemangioma can occur before significant proliferation and may even be present congenitally.

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Citations

Jul 9, 2011·European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery·Michele TorreVincenzo Jasonni
Nov 18, 2006·Journal of the American Academy of Dermatology·Arlene M Ruiz-de-LuzuriagaSarah L Stein
Sep 14, 2007·Clinical Genetics·J H Levin, S G Kaler
Dec 2, 2008·Journal of Pediatric Surgery·Michele TorreVincenzo Jasonni
Nov 22, 2007·Journal of the American Academy of Dermatology·Denise W MetryIlona J Frieden
Apr 6, 2013·Korean journal of pediatrics·Na Yong LeeEun Ae Park
Jan 5, 2008·Stroke; a Journal of Cerebral Circulation·Geoffrey L HeyerPhilip Meyers

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