Sir,
Lymphatic malformation (LM) or lymphangioma is a benign proliferation of the lymphatics accounting for four per cent of all vascular malformations and 26% of all benign vascular tumors.[1,2]
A 26-year-old female presented with vulvar hypertrophy since 10 years of age, which gradually increased in size after puberty. It was associated with episodic pain on and off interfering with walking. There was no history of oozing. Cutaneous examination of the external genitalia revealed gross vulvar hypertrophy extending upto the fourchette. The overlying skin was hyper- pigmented, indurated, rugose and studded with multiple skin colored papules. [Figures 1 and 2] Based on the clinical appearance, a differential diagnosis of vulvar lymphangioma and genital warts was entertained. Biopsy for histopathology revealed dilated lymphatics lined with flattened endothelial cells and luminal macrophages, neutrophils and lymphocytes consistent with our diagnosis of lymphangioma.
Figure 1.
Clinical picture shows vulvar hypertrophy with a rugose appearance
Figure 2.
Close up shows multiple superficial skin colored shiny papules and warty excrescences
LMs are broadly classified into superficial lymphangioma circumscriptum and deeper cavernous lymphangioma.[3] There is no clear distinction between both, the difference being solely on the extent of the malformation.[4] Differentiation between congenital vs. acquired lymphangiomas with respect to localization within the skin have been made. The former result from a hamartomatous malformation of lymphatic vessels, the latter from acquired obstruction of lymph vessels, e.g. after surgical or radiation treatment of malignancies of the breast or uterus.[5] Acquired lymphangioma of the vulva, arising without obvious causes, seems to be unusual.[6]
Lymphangiomas of the vulva are rare. Thirteen cases of the congenital and 24 cases of acquired form have been reported in the literature presently.[7] Diffuse lymphangiomas, though present from birth, may go unnoticed for many years. They present as asymptomatic, erythematous flat indurated or atrophic plaques. Swelling may or may not be apparent. Diffuse lymphangiomas, unlike the superficial type, may not have any surface changes. The diagnosis is usually made by biopsy.[7]
Various modalities of treatment have been suggested viz., observation, surgical excision of skin and subcutaneous tissues, surface ablation with Laser (CO2, Er:YAG), sclerotherapy, superficial radiotherapy.[8–10] Our patient was advised vulvectomy but she was lost for follow-up.
References
- 1.Kim JK, Yoo KS, Moon JH, Park KH, Chung YW, Kim KO, et al. Gallbladder lymphangioma: A case report and review of the literature. World J Gastroenterol. 2007;13:320–3. doi: 10.3748/wjg.v13.i2.320. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Colange E, Wilson JE. Vascular tumors, tumors and tumour like conditions of blood vessels and lymphatics. In: Elder DE, Elenitsas, Johnson BL Jr, Murphy GF, editors. Lever's histopathology of skin. 9th ed. Philadelphia: Lippincott Williams and Wilkins; 2005. pp. 1046–7. [Google Scholar]
- 3.Flanagan BP, Helwig EB. Cutaneous lymphangioma. Arch Dermatol. 1977;113:24–30. [PubMed] [Google Scholar]
- 4.Rao R, Shenoi SD. Diseases of Arteries, Veins and Lymphatics. In: Valia RG, Valia A, editors. IADVL textbook of dermatology. 3rd ed. Mumbai: Bhalani publishing house; 2008. pp. 729–31. [Google Scholar]
- 5.Mehta V, Gupta S, Shenoi D. Acquired lymphangioma of vulva secondary to radiotherapy for carcinoma cervix. Indian J Dermatol. 2003;48:221–2. [Google Scholar]
- 6.Akimoto K, Nogita T, Kawashima M. A case of acquired lymphangioma of the vulva. J Dermatol. 1993;20:449–51. doi: 10.1111/j.1346-8138.1993.tb01318.x. [DOI] [PubMed] [Google Scholar]
- 7.Ghaemmaghami F, Karimi Zarchi M, Mousavi A. Surgical management of primary vulvar lymphangioma circumscriptum and postradiation: Case series and review of literature. J Minim Invasive Gynecol. 2008;15:205–8. doi: 10.1016/j.jmig.2007.09.005. [DOI] [PubMed] [Google Scholar]
- 8.Ochsendorf FR, Kaufmann R, Runne U. Erbium:YAG laser ablation of acquired vulval lymphangioma. Br J Dermatol. 2003;144:442–4. doi: 10.1046/j.1365-2133.2001.04060.x. [DOI] [PubMed] [Google Scholar]
- 9.Ghaemmaghami F, Karimi Zarchi M, Mousavi A. Major labiaectomy as surgical management of vulvar lymphangioma circumscriptum: Three cases and a review of the literature. Arch Gynecol Obstet. 2008;278:57–60. doi: 10.1007/s00404-007-0440-3. [DOI] [PubMed] [Google Scholar]
- 10.Huilgol SC, Neill S, Barlow RJ. CO2 laser therapy of vulval lymphangiectasia and lymphangioma circumscriptum. Dermatol Surg. 2002;28:575–7. [PubMed] [Google Scholar]