Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy

Abstract

Elephantiasis nostras verrucosa is characterized by chronic secondary, non-filarial lymphoedema due to recurrent lymphangitis, dermal fibrosis, and epidermal changes consisting of hyperkeratotic, verrucous and papillomatous lesions. Histologically, there is pseudoepitheliomatous hyperplasia. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes. In this study, rapid disappearance of the hyperkeratotic and verrucous lesions, remarkable flattening of the papillomatous nodules and improvement of lymphoedema occurred in three obese patients treated with etretinate in an initial dose of 0.6-0.75 mg/kg/day for 4-6 weeks. Monitoring of plasma concentrations of etretinate, acitretin and 13-cis-acitretin by HPLC revealed sufficient short-time absorption (4 h) and bioavailability of the drug (30 days; two out of three patients). Long-term maintenance therapy in one patient produced a remarkable improvement in the lymphoedema; another patient relapsed after discontinuation of the etretinate and responded again after this was reintroduced. In the third patient treatment was withdrawn because of an increase in triglycerides, but improvement persisted 6 months later. The clinical side-effects of oral retinoid therapy were moderate and well tolerated.

A Case of Stasis Papillomatosis Associated with Psoriasis Vulgaris

“…Histological findings were also similar to those of reported by Zouboulis (4) and Stoberl (5, 6) as elephantiasis nostras venucosa except for deposits of hemosiderin. Zouboulis (4) suggested that functional incapacity of lymphatics, which are the major escape routes for excess proteins and fluids in tissues, causes a high interstitial protein concentration, causing fluid to be retained in tissues as a result of osmotic forces and resulting in chronic inflammatory changes with fibrosis and epidermal hyperplasia. One of the etiologies reported for this condition is chronic bacterial infection (1)(2)(3).…”

Elephantiasis nostras verrucosa: An institutional analysis of 21 cases

“…Case reports of ENV evolving in the absence of cellulitis or lymphangitis exist. 6 Our study highlights the importance of two lesser appreciated factors in the setting of ENV, specifically obesity and CVI. All 21 patients were obese with 91% of them meeting criteria for morbid obesity (BMI $ 40).…”

“…Both topical and systemic retinoids have been successfully used to mitigate the cutaneous manifestations of ENV. 6,21 Two case reports illustrated favorable results with the use of scalpel debridement. 22,23 In one of these surgical reports, adjunctive dermabrasion with a motorpowered grinder was used.…”

A Case of Elephantiasis Nostras Verrucosa

“…Treatments for ENV include conservative measures to reduce lymphostasis (compressive stockings, mechanical massage), medical management of edema by diuretics, and prevention of recurrent infection by prolonged courses of antibiotics 2 . Oral retinoids may be effective, but the sustainability of these results appears variable 14 .…”