Congenital Lymphangioma Circumscriptum of vulva presenting As Multiple Giant Mass Lesions (A Case Report & Literature Review)

Background: Vulvar Lymphangioma circumscriptum (LC) is a rare benign disorder with cutaneous vesiculo-papular lesions which is mostly acquired following pelvic lymphadenectomy or radiotherapy. It is commonly painless, but psychosexual dysfunction, undesired self- body image, clear exudate, pain and recurrent cellulitis can disturb the patient. It should be differentiated from herpes zoster, molluscum contagiosum, hydradenitis, liomyioma and genital warts. Herein, we present a rare case of vulvar congenital lymphangioma with multiple enlarged masses. Case report: A G2L2 33-year-old woman attended our clinic with large verrucous warty masses in labia major, perinea and end-portion of spines measuring about 15×7cm, 9×7cm and 8×8cm respectively. Notably, her right upper and left lower limbs had gross congenital lymphedema. Since puberty she noticed vesicular lesions which gradually progressed in subsequent years till the past three years which significant enlargement of lesions occurred. This time was concordant with the years following parturitions. Lesser anal lesions had been excised eight years ego, with pathologic report of LC. We removed vulvar masses by superficial partial vulvectomy with a qualified margin without using skin graft. One year follow-up revealed normal healing without any recurrence. Conclusions: Congenital lymphangiomas can be highly triggered by hormonal stimulating situations like puberty and pregnancy. So, it is rather to visit the affected cases timely in order to excise these lesions before massive enlargement. In our experience, a superficial partial vulvectomy without skin graft can be a sufficient procedure. Obviously, appropriate approach during and after surgery would be very important for getting a desirable healing.

Keywords:

lymphangioma circumscriptum , congenital , vulva , superficial partial vulvectomy