Extensive Grouped Papules on the Vulva in a Patient with Cervical Cancer

A 71-year-old female presented with extensive, brownish grouped papules on the vulva for unknown duration [Figure 1a]. Fifteen years ago, she underwent radical hysterectomy and bilateral salpingo-oophorectomy with pelvic lymph node dissection, followed by chemoradiation therapy for cervical cancer. Two years ago, she also underwent lymphaticovenous anastomosis surgery due to bilateral leg lymphedema following radiation therapy; however, lymphedema recurred after the surgery. Recent computed tomographic scan and magnetic resonance imaging showed no evidence of structural abnormality or recurrence of cervical cancer. Histopathological examination revealed multiple dilated channels filled with eosinophilic and amorphous fluid in the upper dermis [Figure 1b], which demonstrated positivity in D2-40 immunohistochemistry [Figure 1c]. Laser ablation was recommended, but she declined further treatments.Figure 1: (a) A 71-year-old female presented with extensive, brownish clustered papules on the vulva for unknown duration. (b) Histopathological examination revealed multiple dilated channels filled with eosinophilic and amorphous fluid on the upper dermis (H and E ×100), which showed (c) positivity in D2-40 immunohistochemistry (D2-40 ×100)What is your diagnosis? Answer: Acquired lymphangiectasia of the vulva Discussion Acquired lymphangiectasia of the vulva (ALV), also known as acquired vulvar lymphangioma circumscriptum, is a rare, benign disease of lymphatic malformation.[1,2] Lymphangiectasia can be classified into primary and acquired forms: The primary form often presents as congenital, large nodules, whereas the localized form typically presents as acquired, small nodules.[1,2] ALV is usually secondary to pelvic lymphatic obstruction due to various origins such as tumours, surgery, radiotherapy, Crohn disease, or tuberculosis.[1] Surgery and radiotherapy may cause postsurgical fibrosis followed by subsequent dilatation of the dermal lymphatics, leading to ALV.[3] ALV clinically present as the scattered or grouped, skin-coloured papules or translucent vesicles mimicking frog-spawn with variable symptoms of pruritus or burning sensation.[4] The most commonly involved area of lymphangiectasia is proximal parts of limbs, and vulva is rarely affected area with a few reported cases commonly manifested as a acquired form.[5] Treatment of ALV is not always required, and may be indicated for the purpose of cosmetic improvement or prevention of complications where surgical excision and other palliative modalities including ablative lasers and sclerotherapy may be applied.[1,2,5] Our case represents typical manifestation of ALV treatment accompanied by lymphedema, and highlights the prolonged latency of ALV, occurring 15 years after hysterectomy and radiotherapy. Our case was interesting in that both ALV and lymphedema showed severe manifestation on the both side of vulva and limb, indicating that lymphatic occlusion may be the primary cause of ALV. In patients with multiple vesiculopapular lesions on the vulva with previous history of surgery or radiation on the pelvis region, the rare diagnosis of ALV should be considered, and skin biopsy may help the definite diagnosis. Learning points ALV is usually secondary to pelvic lymphatic obstruction due to various causes such as surgery, radiotherapy, Crohn’s disease, tuberculosis, or tumours. Clinically, it presents as scattered or grouped, skin-coloured papules or translucent vesicles resembling frog spawn. Treatment is not always required, and may be indicated for the purpose of cosmetic improvement or prevention of complications by surgery or other palliative modalities. ALV should be considered in patients with multiple vesiculopapular lesions on the vulva with previous history of surgery or radiation on the pelvis region. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.