Acquired lymphangioma circumscriptum of the vulva
1. Department of dermatology Hédi Chaker Hospital Sfax Tunisia<br>2. Department of gynecology Hédi Chaker Hospital Sfax Tunisia
Lymphangiomas are rare benign proliferations of the lymphatic system. Acquired lymphangioma circumscriptum of the vulva is induced by impaired lymph flow. We describe a new case of lymphangioma circumscriptum resulting from tuberculosis involving a lymph node. A 45-year-old female presented with swelling of the vulva for the past 8 years. She had a history of multiple left inguinal swellings successfully treated with four antituberculosis drugs. Her vulval swelling did not respond and was diagnosed as acquired lymphangioma circumscriptum. She becomes the fifth reported case of acquired lymphangioma circumscriptum of the vulva with tubercular lymphadenitis. The single antituberculosis treatment is insufficient to control lymphangioma. Ablative methods seem encouraging.
Lymphangiomas are rare benign proliferations of the lymphatic system. There are three generally acknowledged types of lymphangioma, circumscriptum (or capillary), cavernous, and cystic . Lymphangioma circumscriptum (LC) 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. Vulvar involvement by lymphangioma is rare. We describe a new case of LC and present a brief review of the literature.
In January 2007 a 45-year-old woman presented with vesicles, oozing, and pruritis of the vulval region. The symptoms had started 8 years previously with papules, oozing of clear fluid and progressive edema of the both labia. A history of multiple palpable inguinal lymph nodules was present. A first biopsy (3 years earlier) of inguinal nodes showed dermal inflammation with an epithelioid and giant-cell granulomas. The diagnosis of genital and lymph node tuberculosis was proposed. She was then treated successfully with four antituberculosis drugs (isoniazid, rifampicin, ethambutol, pyrazinamide) for 2 months, then continued with two drugs (isoniazid and rifampicin) for 6 months with progressive regression of the inguinal lymph nodes. She was then referred to our department because there was no improvement of the vulvar condition. There was no history of genital ulcer, trauma, or any digestive symptoms.
The clinical examination revealed a diffuse induration of both labia major with overlying papulo-vesicular lesions (Fig. 1 ). General examination findings were unremarkable.
Histologic examination of vulvar lesion revealed multiple dilated lymphatic vessels in the papillary dermis containing few erythrocytes and mostly fibrin; the vessels were lined by a thin wall comprising endothelial cells and an inflammatory infiltrate in the papillary dermis (Figs. 2, 3). The overlying epidermis was partly hyperkeratotic, with thinning of the stratum malpighii. The diagnosis of LC was proposed. She was treated with anti histaminic drugs and emollients. A cryotherapy treatment has been started.
We present a case of acquired LC of the vulva with tubercular lymphadenitis. To the best of our knowledge, only four cases of lymphangiectasis of the vulva with lymph node tuberculosis have been reported in the English literature (Table I). In our patient, untreated tubercular lymphadenitis coupled with scarring led to the obstruction of the vulval lymphatics resulting in lymphangiectases.
The terms acquired LC and lymphangiectasis are used interchangeably in the literature. Some authors combine the two entities [2, 3] and others differentiate their physiopathological features [4, 5]. Different classifications of LC have been proposed. In 1970, Peachey and al. distinguished two main forms: localized and classic . It is most commonly accepted that lymphangiectasis are clinically and histologically indistinguishable from superficial lymphangiomas and develop in areas affected by obstruction or destruction of lymphatic drainage .
Vulvar LC can be asymptomatic , pruritic , burning  or painful . It is an unpleasant but benign condition. For our patient, pruritis was the only symptom. Clinically, LC is characterized by translucent vesicles, which may be scattered or grouped like frog-spawn . Rarely, the lesions can have a firm hyperkeratotic appearance .
Acquired LC is attributed to many conditions . These include congenital and acquired lymphedema associated with infections (filariasis, sexually transmitted diseases, tuberculosis, erysipelas, and lymphogranuloma venereum), Crohn's disease, congenital dysplastic angiopathy, surgical or radiotherapic procedures, trauma, keloids , scleroderma, dermopathy from penicillamine or corticosteroids, and lymphatic obstruction associated with neoplasia.
The traditional treatment of LC, surgical removal, is frequently unsuccessful with rapid relapses and should be reserved for treatment failures . Vaporization with a CO2 laser has been recommended recently as treatment for lymphangioma circumscriptum of the vulva with an acceptable cosmetic result . Therapeutic failures after laser treatment after the initial course of CO2 treatment have already been reported. Our patient refused surgical treatment.
Complications associated with vulval LC include cellulitis and psychosexual dysfunction [5-15]. Followup of patients with lymphedema is recommended because transformation to lymphangiosarcoma may occur in the edematous limbs . Two cases of squamous cell carcinoma [16, 17] and lymphangiosarcoma  arinsing within lymphangioma circumscriptum have been repoted.
References1. Brown JV, Stenchever MA.Cavernous lymphangioma of the vulva. Obstet Gynecol. 1989 May; 73(5 Pt 2):877-9.
2. Landthaler M, Hohenleutner U, Braun-Falco O. Acquired lymphangioma of the vulva palliative treatment by means of laser vaporization carbon dioxide. Arch Dermatol 1990; 126:967-8.
3. G. Gokdemir, I. Kivanc-Altunay, Yoldemirb. Acquired vulvar lymphangiomatosis after cancer therapy. Inter J Gynecol Obstet 2003; 81: 65-67.
4. A. Virgili, M. Corazza. Lymphangioma and lymphangiectasis of the vulva. J Euro Acad DermatoloVenereol 1997; 8: 229-232.
5. Ochsendorf FR, Kaufmann R, Runne U. Erbium:YAG laser ablation of acquired vulval lymphangioma. Br J Dermatol. 2001; 144 :442-4.
6. Peachey RD, Lim CC, Whimster Iw. lymphangioma of the skin: a review of 65 cases. Br J Dermatol 1970; 83:519-27.
7. Akimoto K, Nogita T, Kawashima M. A case of acquired lymphangioma of the vulva. J Dermatol. 1993; 20:449-51.
8. Handfield-Jones SE, Prendiville WJ, Norman S. Vulval lymphangiectasia. Genitourin Med 1989;65:335-7.
9. Sood M, Mandal AK, Ganesh K. Lymphangioma circumscriptum of the vulva. J Indian Med Assoc 1991; 89: 262-3.
10. Fisher I, Orkin M. Acquired lymphangioma (lymphangiectasis). Report of a case. Arch Dermatol. 1970; 101:230-4.
11. Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol 2003; 101: 946-54.
12. Russell B. Lymphangioma circumscriptum and keloids. Br J Dermatol 1951; 63: 158-159.
13. Eliezri YD, Sklar JA. Lymphangioma circumscriptum: review and evaluation of carbon dioxide laser vaporisation. J Dermatol Surg Oncol 1988; 14:357-364.
14. Haas AF, Narurkar VA. Recalcitrant breast lymphangioma circumscriptum treated by UltraPulse carbon dioxide laser. Dermatol Surg 1998; 2:893-895.
15. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976; 94: 473-86.
16. Short S, Peacock C. A newly described possible complication of lymphangioma circumscriptum. Clin Oncol (R Coll Radiol). 1995; 7:136-7.
17. Wilson GR, Cox NH, McLean NR, Scott D. Squamous cell carcinoma arinsing within congenital lymphangioma circumscriptum. Br J Dermatol 1993; 129: 337.
18. King DT, Duffy DM, Hirose FM, Gurevitch AW. Lymphangiosarcoma arinsing from lymphangioma circumscriptum. Arch Dermatol 1979; 115: 969.
19. Heuvel MV, Stolz E, Notowicz A. Lymphangiectases of the vulva in a patient with lymph node tuberculosis. Int J Dermatol 1979; 18:65-6.
20. Begum AA, Parvez S, Hassan S, Setna F. vulval lymphangioma: the cause still a mystery? Aust N Z J Obstet Gynecol 1995; 35: 345-6.
21. Mendiratta V, Harjai B, Sardana K. Tubercular lymphadenitis with lymphangiectases of the vulva. J Euro Acad Dermatol Venereol 2005; 19: 255-269.
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