Lymphangioma Circumscriptum in Vulva with Klippel-Trenaunay Syndrome.

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 1a]. Meanwhile, her swollen right lower limb was covered by a large geographic telangiectasia [Figure 1b]. Histopathology of labium majus lesion revealed large, irregular, and dilated thin-walled lymphatic channels and endothelial vasculatures in the papillary and reticular dermis [Figure 1c]. Immunohistochemistry demonstrated positive staining for D240, mainly in lymphatic channel endothelium [Figure 1d]. Numerous CD31-dilated vessels included lymphatic and endothelial vascular channels [Figure 1e]. Finally, a diagnosis of LC was confirmed.

Figure 1

A 31-year-old female presenting with Klippel-Trenaunay syndrome and lymphangioma circumscriptum. (a) The lesions of lymphangioma circumscriptum. (b) A large geographic capillary stain and hypertrophy over the right lower limb. (c) Skin biopsy of lesions in vulva (original magnification, ×10). (d) D240 staining was positive in some large lymphatic channels (original magnification, ×10). (e) CD31 staining was positive in numerous dilated vessels (original magnification, ×10).

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.

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