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The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature

Didier Bessis 1, 2, * Michèle Bigorre 3 Nausicaa Malissen 2 Guillaume Captier 4 Christine Chiaverini 4 Claire Abasq 5 Sébastien Barbarot 6 Olivia Boccara 7 Emmanuel Bourrat Hassan El Fertit 8 Catherine Eschard Thomas Hubiche Jean-Philippe Lacour 4 Nicolas Leboucq 9 Emmanuel Mahé 10 Stéphanie Mallet 11 Myriam Marque 8 Ludovic Martin 12 Juliette Mazereeuw-Hautier 13 Nathalie Milla 2 Alice Phan 14 Patrice Plantin 15 Marie-Christine Picot 16 Eve Puzenat 17 Valérie Rigau 18 Pierre Vabres 19 Sylvie Fraitag 7 Franck Boralevi 20, 21 
Abstract : BACKGROUND: Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports. OBJECTIVE: We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations. METHODS: A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed. RESULTS: Of the 78 patients included in the study, 56 underwent cranial and brain imaging. Twenty-three of the 56 patients (41%) had abnormal findings, including the following: (1) cranial/bone defect (30.4%), with direct communication with the central nervous system in 28.6%; (2) venous malformations (25%); or (3) central nervous system abnormalities (12.5%). Meningeal heterotopia in 34.6% (9/26) was the most common neuroectodermal association. Sinus pericranii, paraganglioma, and combined nevus were also identified. LIMITATIONS: The partial retrospective design and predominant recruitment from the dermatology department are limitations of this study. CONCLUSIONS: Infants with HCS or HTS are at high risk for underlying neurovascular anomalies. Magnetic resonance imaging scans should be performed in order to refer the infant to the appropriate specialist for management. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.
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Soumis le : mercredi 30 août 2017 - 12:10:07
Dernière modification le : jeudi 20 octobre 2022 - 15:10:07



Didier Bessis, Michèle Bigorre, Nausicaa Malissen, Guillaume Captier, Christine Chiaverini, et al.. The scalp hair collar and tuft signs: A retrospective multicenter study of 78 patients with a systematic review of the literature. Journal of The American Academy of Dermatology, 2017, 76 (3), pp.478-487. ⟨10.1016/j.jaad.2016.08.046⟩. ⟨hal-01579056⟩



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