Elephantiasis nostras verrucosa is a rare cause of chronic lymphedema, arising in the setting of chronic nonfilarial lymphedema caused by bacterial or noninfectious obstruction of the lymphatics.
It presents mostly as grossly edematous and disfigured lower extremities, due to these regions being gravity-dependent, with thickening and cobble-stoning of the skin and corresponding to the most severe form of chronic lymphedema 1. The edema tends to be non-pitting 1.
It carries an increased risk of malignancies, such as the angiosarcoma in the setting of a Stewart-Treves syndrome.
Elephantiasis nostras verrucosa is caused by obstruction to peripheral lymphatics 1. This can occur secondary to processes such as infection, neoplasm, trauma, obesity, and chronic venous stasis 1.
Obstruction of lymphatics results in an accumulation of fluid in the dermis and subcutis, which compromises the skin as a natural barrier to infection and leads to eventual fibrosis 1. This results in frequent skin infections which further obstruct lymphatic flow, worsening the initial problem in a vicious cycle 1.
Although the diagnosis is primarily a clinical one, elephantiasis nostras verrucosa can be investigated with a MR lymphangiography, which can classify the type of lymphedema and its severity 2.
Treatment and prognosis
The cornerstone of management is to treat the underlying cause(s) 1. However, for symptomatic relief, medications such as diuretics, and other measures such as compression stockings, may be employed 1.
- 1. Liaw FY, Huang CF, Wu YC, Wu BY. Elephantiasis nostras verrucosa: swelling with verrucose appearance of lower limbs. (2012) Canadian family physician Medecin de famille canadien. 58 (10): e551-3. Pubmed
- 2. Liu NF, Yan ZX, Wu XF. Classification of lymphatic-system malformations in primary lymphoedema based on MR lymphangiography. (2012) European journal of vascular and endovascular surgery : the official journal of the European Society for Vascular Surgery. 44 (3): 345-9. doi:10.1016/j.ejvs.2012.06.019 - Pubmed