• Anne-Sophie Dutkiewicz, Khaled Ezzedine, Juliette Mazereeuw-Hautier, Jean-Philippe Lacour, Sébastien Barbarot, Pierre Vabres, Juliette Miquel, Xavier Balguerie, Ludovic Martin, Franck Boralevi, Pierre Bessou, Jean-François Chateil, Christine Léauté-Labrèze, and Groupe de Recherche Clinique en Dermatologie Pédiatrique.
• Department of Dermatology and Pediatric Dermatology, Pellegrin Children's Hospital, Bordeaux, France; National Center for Rare Skin Disorders-Institut National de la Santé Et de la Recherche Médicale (INSERM) U1035, Bordeaux Segalen University, Bordeaux, France. Electronic address: annesophie.dutkiewicz@gmail.com.
• J. Am. Acad. Dermatol. 2015 Mar 1; 72 (3): 473-80.

BackgroundUpper facial port-wine stain (PWS) is a feature of Sturge-Weber syndrome (SWS). Recent studies suggest that the distribution of the PWS corresponds to genetic mosaicism rather than to trigeminal nerve impairment.ObjectivesWe sought to refine the cutaneous distribution of upper facial PWS at risk for SWS.MethodsThis was a prospective multicenter study of consecutive cases of upper facial PWS larger than 1 cm² located in the ophthalmic division of trigeminal nerve distribution in infants aged less than 1 year, seen in 8 French pediatric dermatology departments between 2006 and 2012. Clinical data, magnetic resonance imaging, and photographs were systematically collected and studied. PWS were classified into 6 distinct patterns.ResultsIn all, 66 patients were included. Eleven presented with SWS (magnetic resonance imaging signs and seizure). Four additional infants had suspected SWS without neurologic manifestations. Hemifacial (odds ratio 7.7, P = .003) and median (odds ratio 17.08, P = .008) PWS patterns were found to be at high risk for SWS. A nonmedian linear pattern was not associated with SWS.LimitationsSmall number of patients translated to limited power of the study.ConclusionsSpecific PWS distribution patterns are associated with an increased risk of SWS. These PWS patterns conform to areas of somatic mosaicism. Terminology stipulating ophthalmic division of trigeminal nerve territory involvement in SWS should be abandoned.Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

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