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Indian J Dermatol. 2012 Mar-Apr; 57(2): 149–151.
PMCID: PMC3352644

Secondary Lymphangioma of Vulva: A Report of Two Cases


Secondary lymphangiomas or acquired lymphangiomas of vulva represent dilatation of upper dermal lymphatics following damage to previously normal deep lymphatics. They have been reported to occur following various infections, surgeries and radiotherapy which can cause damage to deep lymphatics.Treatment options in the management of secondary lymphangiomas include surgical resection, carbon dioxide laser vaporisation, sclerosing agents etc. We report two cases of secondary lymphangioma of vulva that followed radiotherapy for carcinoma of cervix. Both the patients were treated successfully by surgery.

Keywords: Lymphangioma, carcinoma cervix, radiotherapy, vulva


Secondary lymphangiomas of vulva consist of dilatation of lymphatics following damage to deep lymphatics due to various causes. They have been reported to occur following radiotherapy to carcinoma cervix, tuberculous inguinal adenitis, pelvic and intra-abdominal surgeries, scrofuloderma, Crohn's disease, scarring after keloids, recurrent cellulitis, and filariasis.

Case Reports

Case 1

A 65-year-old female came to our outpatient department with multiple fleshy and vesicular skin lesions in the external genitalia of 10 years duration. She complained of oozing of copious clear fluid wetting her clothes, making her a social recluse. There was occasional pain and itching over the lesions.

She had been diagnosed to have squamous cell carcinoma of cervix 15 years ago for which she underwent a total hysterectomy and multiple sessions of radiotherapy. There was no history of tuberculosis or other pelvic infections.

On examination, multiple vesicles were seen over labia majora and mons pubis with few lesions exhibiting a verrucous appearance [Figure 1]. Labia majora was edematous and thickened. There was no tenderness on palpation. Inguinal lymph nodes were not enlarged. H and E section of the skin biopsy showed hyperkeratotic, acanthotic epidermis, with multiple dilated lymphatics in the superficial dermis that was consistent with a diagnosis of lymphangioma [Figure 2]. The patient was subjected to a total vulvectomy with double V-Y advancement flap for the reconstruction of labia majora. She is under regular follow up for the past 5 years and is recurrence free till date [Figure 3].

Figure 1
Multiple vesicles over labia majora giving a verrucous appearance
Figure 2
H and E section of the skin biopsy showing multiple dilated lymphatics in the superficial dermis (40×)
Figure 3
Five years after total vulvectomy with double V-Y advancement flap with no evidence of recurrence

Case 2

A 44-year-old lady presented with multiple vesicles over her genitalia for the duration of 16 years. The lesions oozed out clear fluid. She had undergone a hysterectomy 17 years back for carcinoma of cervix that was preceded and followed by ten sittings of radiotherapy.

On examination, there were multiple grouped vesicles over the labia majora with discharge of clear fluid [Figure 4]. There was no inguinal lymphadenopathy. All the baseline investigations including ultrasound of the abdomen were normal. Skin biopsy showed hyperkeratotis, irregular acanthosis, and papillomatosis of the epidermis, with the papillary dermis showing many dilated lymphatics filled with pale, vacuolated secretions [Figure 5]. The patient underwent excision with double V-Y advancement flap cover and is on follow up.

Figure 4
Grouped vesicles over labia majora
Figure 5
H and E section of the skin showing dilated lymphatics filled with lymph in the superficial dermis (40×)


Lymphangiomas consist of dilated lymphatic channels resulting from defective lymphatic drainage due to congenital or acquired causes. Congenital lymphangiomas (lymphangioma circumscriptum) represents malformation of deep dermal and subcutaneous lymphatics with secondary dilatation of superficial lymphatics, whereas acquired lymphangiomas or lymphangiectasia represent dilatation of superficial lymphatics, resulting from obstruction of previously normal deep lymphatics. Acquired lymphangiomas are rare and are reported to occur following radiotherapy to carcinoma of cervix,[1] tuberculous inguinal adenitis,[2] intra-abdominal and pelvic surgeries,[3,4] scrofuloderma,[5] scarring after keloids,[6] Crohn's disease,[7] and recurrent cellulitis.[8] The most common clinical presentation is multiple vesicles with oozing of clear fluid (lymphorrhea). Sometimes, the lesions present with a hyperkeratotic appearance mimicking genital warts.[6] Recurrent cellulitis may be the presenting complaint or develop as a complication of lymphangioma.[1,8] In long-standing cases, squamous cell carcinoma may develop.[9]

Lymphangioma of vulva is best diagnosed clinically. Histopathological findings are confirmatory. They are hyperkeratosis and acanthosis of the epidermis with multiple dilated lymphatics in the papillary dermis. Scattered lymphocytes in the stroma may be seen. The lymphatic endothelial cells have been found to be CD31 and D2-40 positive but CD34 negative.[10] The differential diagnosis include lymphangioma circumscriptum, molluscum contagiosum, and genital warts. Investigations such as dermoscopy,[11] lymphangiography, and magnetic resonance imaging have been used.[12] The latter is done to know the extent of involvement prior to therapeutic intervention.

Various treatment modalities have been tried. Surgical resection with reconstruction of vulva is considered to be the preferred treatment option.[13] Labial reduction offers better results. Other treatment options in the management of lymphangiomas include cryosurgery,[1] carbon dioxide vaporization,[9] sclerotherapy with sclerosing agents like OK-432 (picibanil),[14] electrocautery, and radiotherapy. Various lasers such as pulsed dye and Erbium lasers have been tried.[15] Recurrence is a common problem encountered in the management of lymphangiomas. Keloid formation has been reported following carbon dioxide laser therapy.


Source of Support: Nil

Conflict of Interest: Nil.


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