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CASE IN POINT - CLINICS IN DERMATOLOGY
Year : 2016  |  Volume : 3  |  Issue : 3  |  Page : 162-164

Acquired vulvar lymphangioma circumscriptum


Division of Dermatology, National University Hospital, Singapore

Date of Web Publication27-Feb-2017

Correspondence Address:
Dr. Aditi Sobti
Division of Dermatology, National University Hospital
Singapore
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/astrocyte.astrocyte_5_17

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  Abstract 

Lymphangioma circumscriptum is a benign dilatation of lymph channels localized to the skin and subcutaneous tissues. This dermatological condition is generally found localized to the oral mucosa, tongue, proximal parts of arms and legs, groin, axilla, and trunk. Primary vulvar involvement is very rare. This report dwells on a 61-year-old female presenting with multiple itchy translucent warty papules on the left vulva. These lesions had been present for the past 3 years, and were gradually increasing in number and size. She had undergone a hysterectomy for uterine carcinoma 13 years ago, and had subsequently developed lymphedema of the left leg. Histopathologic examination of the biopsied vulvar papule demonstrated hyperkeratotic epidermis and dilatation of thin-walled lymph vessels in superficial dermal papillae. The physiopathological features helped clinch the diagnosis of vulvar lymphangioma circumscriptum.

Keywords: Acquired lymphangioma circumscriptum, dilated lymph vessels, hyperkeratotic epidermis, vulvar lymphangioma circumscriptum


How to cite this article:
Sobti A, Wee DA, Tan KB. Acquired vulvar lymphangioma circumscriptum. Astrocyte 2016;3:162-4

How to cite this URL:
Sobti A, Wee DA, Tan KB. Acquired vulvar lymphangioma circumscriptum. Astrocyte [serial online] 2016 [cited 2023 Oct 4];3:162-4. Available from: http://www.astrocyte.in/text.asp?2016/3/3/162/200999


  Introduction Top


Lymphangioma circumscriptum (LC) refers to abnormal dilatation of lymphatic vessels in deep dermal and subcutaneous layer. The condition is either primary (usually present at birth or develops in early childhood) or secondary (induced by impaired lymph flow). Secondary lymphangioma is also termed acquired lymphangioma and lymphangiectasis. LC can be found on the proximal extremities, trunk, axilla, and oral cavity but also may occur on the penis, scrotum, and the vulva. The clustered vesicles characteristically cover a surface area greater than a square centimeter, and it is uncommon for vesicular areas to form plaques or undergo verrucous changes.


  Case Report Top


A 61-year-old female presented to the dermatology outpatients' clinic with itchy, raised lesions on the left vulva. These lesions had been present for the past 3 years, and were gradually increasing in number and size. She did not have any other similar lesions elsewhere. She denied any history of multiple sexual partners or similar lesions in the husband. Detailed past history revealed she had uterine carcinoma, which was treated by hysterectomy 13 years ago. Following the operation, she developed left leg lymphedema that was reduced by lymphovenous anastomosis 5 years ago.

Clinical examination revealed multiple translucent warty papules on the left vulva [Figure 1]. She had no other significant findings. A differential diagnosis of genital warts, acrochordons, and bowenoid papulosis was considered.
Figure 1: Multiple discrete hyperkeratotic warty papules scattered over the left vulva.

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Histology of the biopsied papule revealed mildly acanthotic epidermis, hyperkeratosis and focal parakeratosis, and dilatation of thin-walled lymphatic vessels in the superficial dermal papillae, with their lumen containing some red blood cells and lymphocytes. Mild chronic inflammatory infiltrate of lymphocytes and plasma cells was found in the dermis with scattered melanophages. Few scattered thick-walled lymph vessels were present in the deeper dermis and subcutis [Figure 2]. There was no evidence of dysplasia or malignancy. These histological findings confirmed the diagnosis of LC.
Figure 2: Skin biopsy histophotomicrograph. Dilated thin-walled lymphatic vessels stand out in the papillary dermis, associated with surrounding lymphocytic infiltrates and overlying epidermal acanthosis (H and E, original magnification ×40).

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Because the patient refused active surgical treatment due to the possibility of recurrence, no operative intervention was carried out, and the patient has been kept under observation.


  Discussion Top


This case of acquired vulvar LC is presented to emphasize that, met with a clinical picture expectant with the possibility of LC, a definite suspicion must be kept if a significant past surgical history exists, which can explain the physiopathology associated with the entity.

Lymphangiomas are rare benign proliferations of the lymphatic system. Acquired LC of the vulva is induced by impaired lymph flow. There are three generally acknowledged types of lymphangioma, namely, circumscriptum (or capillary), cavernous, and cystic.[1],[2]

Different classifications of LC have been proposed. Essentially, two main forms stand distinguished, namely, localized and classic. It is most commonly accepted that lymphangiectasis is clinically and histologically indistinguishable from superficial lymphangiomas and develops in areas affected by obstruction or destruction of lymphatic drainage.[3]

Acquired LC is attributed to injury and damage to the deep collecting channels in the connective tissue leading to the buildup of the lymph in the superficial vessels and subsequent formation of the lesions. Specific to vulvar lymphangiomatosis, the causes can be wide and varied. This may include infections including filariasis, sexually transmitted diseases, tuberculosis, erysipelas, and lymphogranuloma venereum; Crohn's disease of vulva; oncology-related surgical procedures, and radiotherapy mostly undertaken for carcinoma of the uterus, cervix, or vulva; trauma; scleroderma; and dermopathy from penicillamine or corticosteroids.[4],[5]

Clinical presentation ranges from thin-walled vesicles, resembling frog-spawn filled with a clear fluid, to hyperkeratotic papules. The possible explanation for this variation in the morphology is a gradual tissue organization, probably enhanced by the presence of lymphedema.

Clinically, the differential diagnoses can include vulvar warts or condyloma accuminata,[6] epidermal cysts, steatocystoma multiplex, Fox–Fordyce disease, cherry hemangioma, and angiokeratoma.[4] Histolopathologic features of LC include dilated lymph vessels in the upper dermis causing expansion of the papillary dermis; they may also extend into the subcutis. There may be acanthosis and hyperkeratosis of the overlying epidermis.[7]

Though LC is often asymptomatic, vesicles are prone to rupture with weeping and crusting, and a history of recurrent bacterial infection is common. This is particularly true if the lesions are itchy or irritating, as excoriations allow a portal for bacterial entry. Vulvar LC can be asymptomatic, pruritic, burning, or painful. It is an unpleasant but benign condition.

The definitive treatment for LC is a surgical excision with removal of both the superficial and deep components;[8] however, the recurrence rates are high, especially in lesions with deeper involvement. Other palliative treatments include superficial X-ray therapy, radiotherapy, argon laser, CO2 laser, 900-nm diode laser, pulsed dye-laser, and sclerotherapy.[9]

Complications associated with vulvar LC include cellulitis and psychosexual dysfunction. Rarely, hidradenitis suppurativa, squamous cell carcinoma [10],[11] and lymphangiosarcoma may develop within LC. Follow-up of patients with lymphedema is recommended because transformation to lymphangiosarcoma may occur in the edematous limbs.[12]


  Conclusion Top


Vulvar LC is a rare clinical entity which usually presents as thin-walled vesicles, resembling frog-spawn filled with a clear fluid or hyperkeratotic papules. The condition is often asymptomatic but may produce psychosexual dysfunction and sometimes lead to cellulitis, particularly if the lesions are itchy or irritating and provoke excoriations. The treatment is riddled with hardships because surgical removal is frequently unsuccessful and is associated with rapid relapses.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Brown JV, Stenchever MA. Cavernous lymphangioma of the vulva. Obstet Gynecol 1989;73:8779.  Back to cited text no. 1
    
2.
Virgili A, Corazza M. Lymphangioma and lymphangiectasis of the vulva. J Euro Acad Dermatol Venereol 1997;8:229-32.  Back to cited text no. 2
    
3.
Akimoto K, Nogita T, Kawashima M. A case of acquired lymphangioma of the vulva. J Dermatol 1993;20:44951.  Back to cited text no. 3
    
4.
Núñez EC, Peñaranda JM, Alonso MS, Ortiz-Rey JA. Acquired vulvar lymphangioma: A case series with emphasis on expanding clinical contexts. Int J Gynecol Pathol 2014;33:235-40.  Back to cited text no. 4
    
5.
Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: A review of the literature. Obstet Gynecol 2003;101:946-54.  Back to cited text no. 5
    
6.
Sultan A, Dadras SS, Bay JM, Teng NN. Prox-1, Podoplanin and HPV staining assists in identification of lymphangioma circumscriptum of the vulva and discrimination from vulvar warts. Histopathology 2011;59:1274-7.  Back to cited text no. 6
    
7.
Rapini RP. Vascular proliferations and neoplasms. In: Rapini RP, editor. Practical Dermatopathology. 1st ed. St Louis: Elsevier Mosby; 2005. pp. 332.  Back to cited text no. 7
    
8.
Dikmen Y, Coşan Terek M, Akman L, Solmaz U, Zekioğlu O, Akalin T. Congenital vulvar lymphangioma circumscriptum. Int J Clin Oncol 2008;13:458-60.  Back to cited text no. 8
    
9.
Lapidoth M. Treatment of lymphangioma circumscriptum with combined radiofrequency current and 900nm diode laser. Derm Surg 2006;32:790.  Back to cited text no. 9
    
10.
Short S, Peacock C. A newly described possible complication of lymphangioma circumscriptum. Clin Oncol 1995;7:136-7.  Back to cited text no. 10
    
11.
Sims SM, McLean FW, Davis JD, Morgan LS, Wilkinson EJ. Vulvar lymphangioma circumscriptum: A report of 3 cases, 2 associated with vulvar carcinoma and 1 with hidradenitis suppurativa. J Low Genit Tract Dis 2010;14:234-7.  Back to cited text no. 11
    
12.
Landthaler M, Hohenleutner U, BraunFalco O. Acquired lymphangioma of the vulva palliative treatment by means of laser vaporization carbon dioxide. Arch Dermatol 1990;126:9678.  Back to cited text no. 12
    


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