Lymphangioma Circumscriptum (Lymphangiectasia)
Lymphangioma circumscriptum (LC) is a rare but benign condition of dilatation of the dermal lymphatic vessels visible through the epidermis due to obstructed lymphatic return. While skin manifestations are a common presentation, vulvar LC is rare and ofte
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Sarah Shaffer and Colleen K. Stockdale
44.1 Introduction Lymphangioma circumscripta (LC), lymphangiectasia, and superficial lymph vessel ectasia [1] are a few of the terms used to describe a rare but benign condition of dilatation of the dermal lymphatic vessels visible through the epidermis due to obstructed lymphatic return. A vascular malformation of the lymphatics may be superficial, located in the layers of the skin, or may include a deeper component causing an extensive area of grouped and/or multiloculated bulging lymphatic vessels. Congenital malformations or defects can cause LC in children. Acquired LC in adults can have many causes including trauma or localized radiation therapy; if lymphatic obstruction and chronic edema result, LC may develop (Fig. 44.1).
44.2 Clinical Presentation Lymphangioma circumscripta can occur anywhere on the body including intraperitoneal and retroperitoneal manifestations [2]. Skin manifestations are far more common, but vulvar LC is rare. The subepidermal vessels of LC can be mistaken for vasculature, vesicles, papules, angiokeratomas, and genital warts
S. Shaffer · C. K. Stockdale (*) Department of Obstetrics and Gynecology, University of Iowa, Iowa City, IA, USA e-mail: [email protected]
Fig. 44.1 Vulvar lymphangioma. The dilatation of the dermal lymphatic vessels is visible through the epidermis as vesicles or bullae. Courtesy of Professor Jacob Bornstein
[2], among other vulvar pathologies. Color can vary from white to brown to purple and size can range up to 2 cm in diameter. Associated symptoms can include swelling, pain, or a sensation of heaviness. Patients may present after observing the lesions or with weeping of lymphatic fluid from disrupted lesions. The fluid is chylous and may appear milky or may be blood-tinged [1]. The amount of fluid lost from these lesions can be extensive in some situations. The lesions of LC, if disrupted, can become eroded and ulcerated and may become infected causing a secondary cellulitis. The appearance, the symptoms, or
© Springer International Publishing AG, part of Springer Nature 2019 J. Bornstein (ed.), Vulvar Disease, https://doi.org/10.1007/978-3-319-61621-6_44
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complications of vulvar LC can lead to alteration of sexual activity and psychosocial dysfunction [2].
44.3 Pathogenesis History most often will elucidate a prolonged presence of the lesions with minimal symptoms. Considering causes of acquired vulvar LC, the most common predisposing condition in one review was radical surgery and/or radiation for cervical cancer and Hodgkin’s lymphoma [2–4]. Malignancy-associated LC can demonstrate significant delay to onset [5] and may be accompanied by lymphedema of adjacent body regions causing a large amount of chylous discharge from the LC [1]. There are reports of LC related to fistulous Crohn’s disease and tuberculous infection [3] as well as rare reports of pregnancy-induced LC [1, 3]. In addition to a careful history and physical examination, a biopsy may be
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