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PHACE syndrome, a series of six patients: clinical and morphological manifestations, propranolol efficacy, and safety.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Bellaud, Guillaume Puzenat, Eve Billon-Grand, Nora Célia Humbert, Philippe Aubin, François
Description	Country affiliation: France Author Affiliation: Bellaud G ( Department of Dermatology, University Hospital Jean Minjoz, Besançon, France.)
Abstract	INTRODUCTION: PHACE syndrome is defined by infantile hemangiomas associated with a spectrum of malformations. These abnormalities involve posterior fossa of the brain, cerebral vasculature, heart or aorta, eyes, and midline thorax or sternum. CASE REPORTS: Six cases of PHACE syndrome were diagnosed between 2002 and 2011 in the Department of Dermatology at Besançon Hospital. All patients were female and had an infantile hemangioma of the face over 5 cm in size. Five of them had encephalic abnormalities, and arterial malformations were found in three patients. Only one patient showed an ocular abnormality. None of them presented any cardiac, aortic, or sternal malformations. Two patients benefited from treatment with propranolol with good effectiveness and safety. DISCUSSION: The presence of an infantile hemangioma over 5 cm in size on the face should be a suspected sign for PHACE syndrome and requires the completion of a systematic evaluation. Diagnosis is based on recently revised criteria and demonstrates the large polymorphism of the malformations that may be encountered. CONCLUSION: This descriptive series is in accordance with literature data including female preponderance and the frequent involvement of the posterior fossa. It also underlines the effectiveness of propranolol for this indication.
File Format	HTM / HTML
ISSN	00119059
Issue Number	1
Volume Number	54
e-ISSN	13654632
Journal	International Journal of Dermatology
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2015-01-01
Publisher Place	Great Britain (UK)
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Neurocutaneous Syndromes Adverse Effects Complications Humans Drug Therapy Aortic Coarctation Child, Preschool Infant Discipline Dermatology Therapeutic Use Journal Article Neoplastic Syndromes, Hereditary Case Reports Vasodilator Agents Eye Abnormalities Skin Neoplasms Female Diagnosis Facial Neoplasms Propranolol Hemangioma, Capillary Child Infant, Newborn
Content Type	Text
Resource Type	Case study Article
Subject	Dermatology

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