title: Lymphangioma circumscriptum of the vulva in a patient with Noonan syndrome.
abstract: Lymphangioma circumscriptum (LC) results from the development of abnormal lymphatic vasculature and is characterized by the presence of grouped vesicles filled with clear or colored fluid. Vulvar localization is uncommon. Abnormalities of the lymphatic system, such as lymphedema and cystic hygroma, are well-known features of genetic disorders such as Noonan syndrome (NS) and Turner syndrome. We report the case of a patient with NS who presented with LC of the vulva. We also discuss the expanding spectrum of clinical anomalies associated with the presentation of NS.
authors.affiliation: Division of Dermatology, Ohio State University Medical Center, 2012 Kenny Rd, Columbus, OH 43221, USA. Llana.Pootrakul@osumc.edu.
authors.forename: Llana
authors.initials: L
authors.lastname: Pootrakul
authors.forename: Michael R
authors.initials: MR
authors.lastname: Nazareth
authors.forename: Richard T
authors.initials: RT
authors.lastname: Cheney
authors.forename: Marcelle A
authors.initials: MA
authors.lastname: Grassi
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques / Q000379:methods; D008202:Lymphangioma / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D009634:Noonan Syndrome / Q000150:complications*; D014845:Vulvar Diseases / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D014846:Vulvar Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*