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Case Report | Volume 19 Issue 2 (April-June, 2026) | Pages 18 - 22
Lymphangioma Circumscriptum of the Face: A Rare Entity
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1
Dermatology Department, Ministry of the National Guard - Health Affairs, Jeddah, Saudi Arabia. King Abdullah International Medical Research Center, Jeddah, Saudi Arabia. King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
2
King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
Under a Creative Commons license
Open Access
Received
March 24, 2026
Revised
April 2, 2026
Accepted
April 2, 2026
Published
April 6, 2026
Abstract

Lymphangioma circumscriptum, also known as microcystic lymphatic malformation, is a rare benign lymphatic anomaly that typically presents in early childhood and most commonly involves the trunk, axillae, and genital regions. Facial involvement is uncommon and may pose diagnostic challenges due to its atypical location and resemblance to other vesiculobullous conditions. A 14-year-old female presented with an 8-year history of asymptomatic vesicular lesions localized to the right zygomatic region. Examination revealed grouped translucent vesicles with a frogspawn appearance. Histopathologic evaluation showed dilated dermal lymphatic channels with lymphoid aggregates, and immunohistochemistry was positive for D2-40, confirming the diagnosis. This case highlights a rare facial presentation of lymphangioma circumscriptum and underscores the importance of considering lymphatic malformations in the differential diagnosis of chronic vesicular facial lesions. Histopathologic and immunohistochemical evaluation remains essential to ensure accurate diagnosis and appropriate management.

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