Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature

Abstract

In any acquired or clinically unusual lymphatic process in the skin and soft tissues, a lesion or obstructive process of the draining lymphatics should be investigated.

Localized acquired lymphangiectasias after breast surgery: Enhanced non‐invasive diagnosis using dermoscopy and reflectance confocal microscopy

“…Acquired lymphangiectasias have been reported in the literature with an increased frequency in the past years due to increase in surgery for certain malignancies involving breast, male and female genitalia, and/or other internal organs …”

“…They clinically appear as clusters of asymptomatic, translucent, and flesh‐colored vesicular lesions; some of them may turn purple due to the presence of red blood cells . Lymphangioma circumscriptum is the most common congenital form whereas acquired lesions, known as lymphangiectasias, resulting from damage and/or obstruction of previously normal lymphatics, usually develop secondary to surgery, radiotherapy, and/or infections . In this case, clinical presentation may range from few, isolated vesicles measuring 2‐10 mm in diameter on normal‐appearing skin, to several, clustered, sometimes verrucous, lesions associated with diffuse lymphedema .…”

Vulvar lymphangioma circumscriptum: comparison of primary and acquired forms in a cohort of 57 patients

“…Median age at diagnosis for acquired VLC was 52 years, similar to that previously reported and Chang's series (50-51 years), older than for primary VLC patients. 6,7 The interval from lymphoedema-symptom onset to first specialized consultation was shorter for acquired than primary VLC, probably because oncologists are well aware of the risk of lymphoedema after some cancer treatments, are sensitive to it and promptly referred patients to specialized lymphoedema-dedicated centres, whereas primary-care physicians are apparently less adequately trained to recognize this disease. 8 Sometimes, VLC is also confounded with warts or condyloma acuminata.…”

“…10 Other treatments were also proposed in the literature, such as yttrium-aluminium-garnet laser, cryotherapy or sclerotherapy, CO 2 laser. 6 VLC is a very rare disease, causing major discomfort, aesthetic prejudice and risk of infection. Primary-care physicians and specialists need to be made aware of this entity's existence so that it can be recognized more rapidly, especially primary VLC, and the patients referred to a specialist for diagnosis confirmation, providing patients with explanations about their disease and its complications, and proposing appropriate therapeutic interventions.…”

Primary lymphangioma circumscriptum of the vulva presenting as warty plaques

“…Differential diagnoses includes molluscum contagiosum, irritant contact dermatitis and tuberculosis verrucosa cutis. If associated with significant hyperkeratosis, the clinical presentation may mimic condyloma acuminata . A high index of clinical suspicion is thus imperative, especially in cases with atypical presentations.…”