Lymphangiectasias of vulva

Sir,

Acquired Lymphangiomas (lymphangiectasias) are benign proliferations of lymphatic system.[1] When congenital or presenting in early childhood they are also called lymphangioma circumscriptum. Lymphangiectasias also somewhat erroneously calledr lymphangiectasias, present later in life as a consequence of impaired lymphatic flow due to disease, surgery or radiation.[1] Lymphangiectasias share clinical and histological features with lymphangioma circumscriptum.[2,3] Lymphangiectasias of vulva is very rare and there are less than 30 reports in the literature.[4] Moreover, this entity poses a diagnostic and therapeutic challenge in view of the difficult anatomical site.[3,5]

A 50-year-old female presented with asymptomatic, persistent, multiple blisters on swollen vulva since the last 3 months. She had undergone hysterectomy, lymph node dissection and subsequent irradiation for cervical cancer 12 years ago.

On examination, the patient had bilateral, multiple, polysized, translucent vesicles over labia majora resembling “frogspawn” [Figure 1] with clear, watery, non-foul smelling discharge in a few areas. Vulval edema was present but there was no pedal edema. Abdominal and per vaginal pelvic examination was normal. There was no regional lymphadenopathy. Histopathology of the lesions revealed large dilated lymphatics lined by normal endothelium throughout the dermis [Figures 2 and 3]. Abdominal ultrasonography did not reveal any abnormality. Other routine investigations were normal and HIV was non-reactive. A diagnosis of “acquired lymphangioma of vulva” was made based on the typical clinical picture and histopathology features.[4] Therapeutic options and prognosis were explained to the patient, but she was unwilling to undergo any of the suggested interventions.

Figure 1.

Multiple, polysized, translucent vesicles over labia majora, resembling “frogspawn”

Figure 2.

Dilated lymphatics throughout the dermis (H and E, ×10)

Figure 3.

Large dilated lymphatics lined by normal endothelium in the dermis (H and E, ×45)

Lymphangiectasias of vulva can occur due to trauma, surgery, radiation, pregnancy, keloids, scleroderma, Crohn's disease, malignancies or infections like tuberculosis, filariasis, erysipelas and lymphogranuloma venereum.[3,5] These conditions can cause destruction of lymph nodes and impaired lymphatic flow leading to accumulation of lymph fluid in dermal lymphatics in the form of vesicles.[1] Interestingly, our patient had undergone hysterectomy, lymph node dissection followed by radiotherapy for cervical cancer 12 years ago, which probably led to vulvar lymphangioma more than a decade later.[4,5]

The most common presentation of acquired lymphangioma is the appearance of thin-walled vesicles filled with a clear fluid resembling “frogspawn” as seen in our case.[4] Smooth, flesh colored nodules can also occur. Rarely, they can develop hyperkeratotic surface mimicking warts.[3,4]

Diagnosis and treatment of these vesiculobullous lesions is important because they may be associated with pain, chronic oozing and infection, occasionally leading to cellulitis. The diagnosis is mainly clinical, aided by histopathological finding of dilated lymphatics in the dermis.[4] Treatment is aimed at reduction of underlying lymphedema and control of infection. Daily compression bandage yields good results, but such a measure is difficult in sites like vulvar lymphangioma. Other treatment modalities such as electrodessication, sclerotherapy, cryotherapy, LASER therapy, and surgical excision can also be employed.[1,3] The treatment is relatively simple, though recurrences are not uncommon.

There are less than 30 reports of this rare entity in the literature and very few from India.[4,5] Awareness of this condition is desirable, especially in view of potential misdiagnosis as warts, molluscum contagiosum, herpetic infections or even malignancy.[1,4]