Sternal malformation/vascular dysplasia syndrome with linear hypopigmentation.
We report a 7-year-old boy who presented with a facial haemangioma, a circumscribed depression over the sternum, coarctation of the aorta, ventricular septal defect and dysplastic cerebral arteries responsible for an episode of acute infarct. This combination of clinical features has been described as the sternal malformation/vascular dysplasia syndrome or PHACES syndrome. At the age of 5 years, lines of hypopigmentation were noted on the right arm, the right hand and the back, along the lines of Blaschko, with no history of any preceding inflammatory changes, and have persisted unchanged. These pigmentary changes have not previously been reported in association with this syndrome.
Mazereeuw-Hautier
J
J
Department of Dermatology, Great Ormond Street Hospital, London WC1N 3JH, UK. mazereeuw-hautier.j@chu-toulouse.fr
Syed
S
S
Harper
J I
JI
D000015:Abnormalities, Multiple; D001017:Aortic Coarctation; D002339:Carotid Arteries / Q000002:abnormalities; D002536:Cerebral Arteries / Q000002:abnormalities; D002675:Child, Preschool; D005153:Facial Neoplasms; D006345:Heart Septal Defects, Ventricular; D006391:Hemangioma; D006801:Humans; D017496:Hypopigmentation; D007223:Infant; D008297:Male; D013249:Sternum / Q000002:abnormalities*; D013348:Subclavian Artery / Q000002:abnormalities; D013577:Syndrome