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Lymphangioma Circumscriptum in Vulva with Klippel-Trenaunay Syndrome.

Byline: Xiao-Yang. Liu, Si. Zhang, Heng. Zhang, Jun. Jia, Lin. Cai, Jian-Zhong. Zhang

To the Editor: Lymphangioma circumscriptum (LC) is not a tumor but rather a congenital malformation of the superficial lymphatics. It presents as groups of deep-seated, vesicle-like papules, resembling frog spawn, at birth or shortly thereafter. Also, the sites of predilection are the abdomen, axillae, genitalia, and mouth, particularly the tongue.[1] Klippel-Trenaunay syndrome (KTS) is relatively rare as a congenital vascular anomaly classically defined as the triad of vascular stain, venous varicosities, soft tissue, and/or bony hypertrophy. Many cases of KTS are mixed malformations with capillary, lymphatic, and venous elements.[2] Here, we reported a Chinese woman with vulvar LC coexisting with KTS in the same side of body.

A 31-year-old female, who had been diagnosed as KTS, presented with oozy, painful, and chronic progressive lesions over the right labium majus that had been present for more than 4 years. She was once treated with laser in local hospital, but the lesions were soon recurrent and grew quickly when she was pregnant. According to the dermatological examination, there was a group of red-purple colored, verrucous vesicles varied in size from 2-10 mm with white or yellow secretion, which resembled frog spawn over the right swollen labium majus [Figure 1]a. Meanwhile, her swollen right lower limb was covered by a large geographic telangiectasia [Figure 1]b. Histopathology of labium majus lesion revealed large, irregular, and dilated thin-walled lymphatic channels and endothelial vasculatures in the papillary and reticular dermis [Figure 1]c. Immunohistochemistry demonstrated positive staining for D240, mainly in lymphatic channel endothelium [Figure 1]d. Numerous CD31-dilated vessels included lymphatic and endothelial vascular channels [Figure 1]e. Finally, a diagnosis of LC was confirmed.{Figure 1}

LC is one of the most common types of lymphangiomas. According to the etiology, LC can be divided into congenital and acquired forms. The acquired LC occurs more commonly in the vulvar region.[3] In addition, a report of KTS showed that the presence of geographic vascular stain was a predictor of having lymphatic malformation in KTS patients.[2] Yoon et al .[4] mentioned that there was a relationship between development of cavernous lymphangioma and sex hormone (estrogen and progesterone) levels. That might be a reason why the lesion size of our patient rapidly increased during her pregnancy, while static pressure caused by pregnancy is also a risk factor. The treatments of LC include surgical excision, laser therapy, sclerotherapy, and cryosurgery. Yoon et al .[5] suggested that surgical treatment could provide a more long-lasting answer compared to other treatment modalities, and surgical treatment was especially beneficial to lesions fulfilling criteria of large, deep, distressing symptoms, and treatment failure after nonsurgical treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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There are no conflicts of interest.


1. James WD, Berger TG, Elston DK, editors. Andrews' Diseases of the Skin Clinical Dermatology: Dermal and Subcutaneous Tumors. 11th ed. Philadelphia, PA: Elsevier Inc.; 2011. p. 579.

2. Maari C, Frieden IJ. Klippel-trenaunay syndrome: The importance of 'geographic stains' in identifying lymphatic disease and risk of complications. J Am Acad Dermatol 2004;51:391-8. doi: 10.1016/j.jaad.2003.12.017.

3. Sinha A, Phukan JP, Jalan S, Pal S. Lymphangioma circumscriptum of the vulva: Report of a rare case. J Midlife Health 2015;6:91-3. doi: 10.4103/0976-7800.158968.

4. Yoon KR, Mo AJ, Park SH, Na YS, Park ST. Carvenous lymphangioma of the vulva. Obstet Gynecol Sci 2015;58:77-9. doi: 10.5468/ogs.2015.58.1.77.

5. Yoon G, Kim HS, Lee YY, Kim TJ, Choi CH, Kim BG, et al. Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum. Arch Gynecol Obstet 2016;293:157-62. doi: 10.1007/s00404-015-3801-3.
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Title Annotation:Correspondence
Author:Liu, Xiao-Yang; Zhang, Si; Zhang, Heng; Jia, Jun; Cai, Lin; Zhang, Jian-Zhong
Publication:Chinese Medical Journal
Article Type:Letter to the editor
Date:Apr 1, 2018
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