Acquired lymphangiectasia ('lymphangioma circumscriptum') of the vulva: a report of eight cases.

AIMS: To present the clinico-pathological findings of eight cases of acquired vulval lymphangiectasia (AVL) with discussion of the terminology and differential diagnosis.

METHODS

Vulvectomy or biopsy specimens from eight patients with AVL were reviewed. All patients had undergone surgery, lymphadenectomy and/or radiotherapy, most commonly for carcinoma of the cervix, up to 26 years prior to presentation with the lymphangiectatic lesions. Immunohistochemistry for CD31, CD34, D2-40, p53 and p16 was performed in each case.

RESULTS

The original clinical and pathological diagnoses were most frequently 'lymphangioma circumscriptum' but viral infection was considered in some cases. All specimens showed dermal lymphangiectasia associated with marked reactive epidermal hyperplasia. The lymphatic endothelial cells showed CD31 and D2-40 expression but CD34 was negative. The keratinocytes showed focal p53 immunoreactivity in four cases.

CONCLUSIONS

AVL is the preferred nomenclature for the lesions presented herein. The clinical and histological features usually are characteristic but the differential diagnosis may include condyloma and differentiated type vulval intraepithelial neoplasia (VIN). Immunohistochemistry may be helpful but lack of CD34 expression should be noted and may prove useful in the differential diagnosis of other vulval vascular lesions. Focal p53 protein immunoreactivity should not be considered indicative of differentiated type VIN in this clinical setting.