A 35-year-old female presented with a history of multiple raised lesions over the vulva of 1 year duration. She was asymptomatic initially, later associated with foul smelling discharge and pain. She had similar lesions over the left upper thigh. She was otherwise healthy and there was no history of constitutional symptoms. Thirty three years ago, she was treated for multiple swellings that developed over the right side of her neck and inguinal regions on both sides. Treatment history was suggestive of antituberculosis treatment. She did not undergo any surgery or radiation therapy in the past. Her menstrual cycles were regular. She is married and has four healthy children.
On local genital examination, there was a large tender polypoidal growth measuring around 10 x 6 cm involving both sides of the vulva including bilateral labia majora, clitoris, labia minora, and anterior fourchette with clear, foul smelling discharge in a few areas [Figure 1]. The lesion was firm to hard in consistency. There were few grouped papules of similar morphology over the left upper thigh and over pubic area [Figure 2]. Vulval edema was present. Multiple linear scars were present over the upper thighs and groins bilaterally. There was no regional lymphadenopathy. Complete analysis on blood, liver and renal function tests, urinalysis, and stool examinations were within normal limits. Gynecological workup and ultrasonography of the pelvic organs revealed no abnormality. Mantoux test was negative. Screening tests for human immunodeficiency virus and hepatitis B virus were nonreactive. The patient underwent simple vulvectomy and excision of left thigh growth under spinal anesthesia. Histopathological examination of the vulvectomy specimen revealed thin-walled and ectatic lymphatic channels in the superficial dermis. The dermal papillae and the dermis showed proliferating, congested capillary sized blood vessels, melanophages, and diffuse as well as perivascular mixed inflammatory infiltrate [Figure 3]. Section from the papillomatous growth over left medial thigh showed a similar picture. With the above findings, we came to a diagnosis of lymphangiectasia of vulva.