Vulval elephantiasis

A 45-year-old woman presented with swelling in the genital region since seven years. On examination, she was found to have elephantiasis involving her left leg, not associated with any symptoms. She gave a history of treatment for filariasis in the past. Genital examination revealed a pendulous swelling arising from the left labia mimicking a scrotum with buried penis [Figure 1]. The right side was not involved [Figure 2]. Genital elephantiasis due to filariasis is very rare accounting for only 1–2& of filarial cases.[1]

Figure 1.

Huge pendulous swelling involving the left labia

Figure 2.

Right side is not involved

The term “elephantiasis” was first described by Celsius and was originally used to describe an elephant like appearance of the legs. Later the term was used to describe similar enlargement of arm, chest, breast, penis, scrotum, and vulva.[2] Genital elephantiasis is characterized by enlargement of genitals due to lymphatic obstruction resulting from a multitude of causes. Common causes include lymphogranuloma venereum, donovanosis, tuberculosis, and filariasis. Radical hysterectomy, pelvic lymphadenectomy, irradiation, tumour invasion, direct trauma are less common causes. Metastatic Crohn's disease rarely presents as genital lymphoedema.[3]

Filariasis in most cases results from infection with Wuchereria bancrofti (98%) and the remaining due to Brugia malayi (2%). Elephantiasis is a chronic manifestation of filarial infection, secondary to lymphatic involvement. The death of adult worms provokes acute inflammation and lymphatic dysfunction leading to permanent obstruction of lymphatic channels, lymph stasis and stimulation of fibroblasts resulting in lymphedema and elephantiasis. Filarial elephantiasis mostly involves lower extremities, and vulval elephantiasis is a very rare presentation.