John Libbey Eurotext

European Journal of Dermatology


Port-wine stains are more than skin-deep! Expanding the spectrum of extracutaneous manifestations of nevi flammei of the head and neck Volume 22, issue 2, March-April 2012


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Department of Otolaryngology,, Institute of Pathology,, Department of Dermatology, Philipp University of Marburg Baldingerstr, 35043 Marburg, Germany
  • Key words: capillary malformations, nevus flammeus, port-wine stains, mucosal vascular malformations
  • DOI : 10.1684/ejd.2012.1653
  • Page(s) : 246-51
  • Published in: 2012

It is well known that port-wine stains of the upper part of the face may herald abnormalities of the brain or eye in the form of Sturge-Weber syndrome. This study focuses on other extracutaneous anomalies in patients with nevi flammei of the head and neck, giving rise to functional complications. Patients and methods. A retrospective study was performed on patients with port-wine stains involving the head and neck area. Records were reviewed for demographic parameters, extent of the lesion, clinical complications, diagnostic measures, previous treatments, ultimate therapeutic approach, and outcome. Results. Nine patients, mean age 50.4 years, with port-wine stains and clinical symptoms due to extracutaneous involvement, were admitted and treated from 2006 to 2009. Major clinical features included macrocheilia in three cases, gingival bleeding in two, dysphonia with globus sensation, painful parotideal swelling with recurrent otitis, painful lingual swelling, recurrent epistaxis, and nasal obstruction in one case each. Cases with lower lip hypertrophy were treated by conventional surgical approaches. Recurrent epistaxis and nasal obstruction due to affected inferior turbinate were treated by Nd:YAG laser therapy, and globus sensation and dysphonia by speech therapy. Patients with gingival affection and recurrent otitis were treated by local ear care. Conclusion. Port-wine stains in the head and neck may develop extracutaneous manifestations causing severe problems. A multimodal and interdisciplinary approach is mandatory for an appropriate treatment.