Lympangiectasia of the vulva accompanying congenital lymphedema

Lympangiectasia of the vulva accompanying congenital lymphedema
April 2009

M Ihsan Okur1, Rüştü Köse2, A Mustafa Yıldırım1, Bengü Çobanoğlu3

Dermatology Online Journal 15 (4): 13 1. Assistant Professor, Department of Plastic and Reconstructive Surgery, Fırat Medical Center, Firat (Euphrates) University, Elazig, Turkey2. Assistant Professor, Department of Plastic and Reconstructive Surgery, Harran University Medical Center, Şanlıurfa, Turkey. rkose@harran.edu.tr3.
Assistant Professor, Department of Pathology

Abstract

Lymphangiectasia is a benign condition with multiple dilated lymph vessels in the dermis. Vulvar localization is rare and mostly depends on disturbing the lymphatic flow. We present a patient with vulvar lesions and ipsilateral congenital lower extremity lymphedema. Surgical excision eliminated the lymphangiectasia and improved the appearance of the edematous vulva.

Introduction

Vulvar lymphangiectasia is not usually seen as a result of congenital lymphedema. Lymphangioma circumscriptum, a subtype of lymphangioma, is mainly a congenital malformation that presents in infancy but may be seen at any age. An acquired lesion, especially one related to the obstruction of lymphatic circulation, is defined as lymphangiectasis; however it is histologically indistinguishable from lymphangioma circumscriptum.
Lymphangiectasia of the vulva is a rare disease and usually reported following surgery and radiotherapy, performed because of a genital malignant tumor [1]. Long-standing Crohn disease with fistulae and tuberculosis lymphadenitis are the other associated conditions [1, 2, 3, 4]. Primary lymphedema is an unusual cause of vulvar lymphangiectasia. We present a case of vulvar lymphangiectasia with ipsilateral congenital lower extremity lymphedema.

Case Report

A 27-year-old woman was referred for treatment of a skin lesion on her right labium majus. The plaque had appeared initially at the age of 19. Sometimes vesicles arose on the surface from which clear and occasionally bloody fluid oozed. She underwent a few bouts of vulvar cellulitis. Four years prior, she had been seen in another dermatology clinic and was given the clinical diagnosis of "infected lymphangioma of the vulva," which was treated with antibiotics. Congenital lympedema along the entire right leg was noted. The lymphedema (Fig. 1) was apparent soon after birth and she regularly used a pressure garment. Genital examination revealed a red and skin-colored plaque with shining, clustered papules on the right labium majus and ipsilateral vulvar edema (Fig. 2). This edema deformed the appearance of the vulva, which was the main complaint of the patient. The growth was excised, including the edematous subcutaneous tissue, under general anesthesia. A negative pressure drain was placed and tissues were sutured.

The histopathological examination of the excised tissue showed many thin-wall lumenal structures with irregular size and shape, lined by a monolayer of endothelium in the upper dermis (Fig. 3). Postoperative recovery was uneventful and the surgical wound healed well.

After the surgery, the symmetry of the vulva was improved. Although a thin scar remained along the labium majus, the aesthetic outcome was good (Fig. 4) and the patient was satisfied. The patient was followed up for 31 months. Recurrence and infection were not observed during this period.

Comment

Vulvar lymphangiectasia is mainly associated with obstruction of the lymphatic flow. The lesions typically arise approximately 7-15 years after lymph node dissection and radiotherapy of the genitalia [1, 5, 6, 7, 8]. De novo acquired lesions without lymphatic disturbance are less frequently seen. In some cases, lymphangiectasia appears following lymphedema of the vulva or lower extremity [3, 4, 5, 6, 9]. However, the condition may arise without adjacent lymphedema [3].

Although deep lymphangiomas are seen in primary lymphedema cases, reports detailing subsequent lymphangiectasia of vulva are very few [2, 10]. Buckley and Barnes report a 35-year-old patient who had lymphedema since the age of 14 and underwent two cellulitis attacks. Thereafter, lymphangiectasia on the vulva appeared in the same year [10]. Another patient with late onset lymphedema in the right leg also presented with lymphangiectasia on the labium majus [2]. The lesions emerged three years after the lymphedema.

The mechanism behind development of the vulvar lymphangiectasia in our patient accompanied by congenital lymphedema is probably similar. A 13-year-old boy has been described who presented exhibited scrotal lymphangiectasia with congenital contralateral leg lymphedema [11].

He also experienced recurrent scrotal infections after the lymphangiectasia appeared. Our patient had congenital lymphedema and suffered episodes of cellulitis before and after the vulvar lymphangiectasia appeared.

Lymphangiectasia may be actually expected in primary lymphedema patients because lymphatic obstruction is an important etiologic factor in its etiology. The cause of the higher rate in acquired lymphatic damage may be because of the rapidly increasing pressure when there is a sudden blockage of the superficial lymphatic system. In addition, recurrent cellulitis further damages lymphatic vessels, increases lymphatic pressure, and induces formation of lymphangiectasia in lymphedema patients.

Lymphangiectasia on the vulva can be easily misdiagnosed, especially as viral warts [1-5, 7, 8, 12]. Oozing vesicules or papules and recurrent infections should bring into mind the possibility of lymphangiectasia.

Excisional surgery and carbon dioxide laser are two major treatment modalities in addition to cryotherapy, electrocoagulation, and sclerosing agent injection [13]. Whereas carbon dioxide laser only vaporizes the lesion superficially, it may seal the underlying lymph vessels and diminish recurrence [9, 14]. The laser therapy can be repeated if any recurrences occur. Delayed wound healing, scars, and even keloid formation may be seen after laser therapy [1, 14]. Excisional surgery eliminates the abnormal subcutaneous lymph vessels and cisterns, and corrects of the aesthetic appearance of the edematous vulva. It is the only plausible choice in the therapy of advanced disease [13].

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