title: PHACE syndrome, a series of six patients: clinical and morphological manifestations, propranolol efficacy, and safety.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Dermatology, University Hospital Jean Minjoz, Besançon, France.
authors: Guillaume
authors: G
authors: Bellaud
authors: Eve
authors: E
authors: Puzenat
authors: Nora Célia
authors: NC
authors: Billon-Grand
authors: Philippe
authors: P
authors: Humbert
authors: François
authors: F
authors: Aubin
mesh_terms: D001017:Aortic Coarctation / Q000175:diagnosis / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000175:diagnosis / Q000188:drug therapy*; D005153:Facial Neoplasms / Q000150:complications / Q000188:drug therapy*; D005260:Female; D018324:Hemangioma, Capillary / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009386:Neoplastic Syndromes, Hereditary / Q000150:complications / Q000188:drug therapy*; D020752:Neurocutaneous Syndromes / Q000175:diagnosis / Q000188:drug therapy*; D011433:Propranolol / Q000009:adverse effects / Q000627:therapeutic use*; D012878:Skin Neoplasms / Q000150:complications / Q000188:drug therapy*; D014665:Vasodilator Agents / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents; D011433:Propranolol