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Lymphangioma circumscriptum of the vulva in a patient with Noonan syndrome
Llana Pootrakul1, Michael R Nazareth, Richard T Cheney
1Division of Dermatology, Ohio State University Medical Center, 2012 Kenny Rd, Columbus, OH 43221, USA. Llana.Pootrakul@osumc.edu.
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.
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