Elephantiasis Nostras Verrucosa: A Treated Case With Dietary Intervention and Acitretin.

Abstract

Elephantiasis nostras verrucosa (ENV) is a rare and extreme complication of chronic non-filarial lymphedema. It can lead to severe disfiguration of body parts, especially the lower extremities, and is characterized by non-pitting edema and papulonodules with a verrucose or cobblestone-like appearance. Obesity is a risk factor of ENV. Various treatments have been reported for ENV. A 52-year-old woman presented to our outpatient dermatology clinic with non-pitting edema, cobblestone-like papulonodules, and erythematous plaques on both legs for 2 years. Her medical history included morbid obesity with a body mass index of 44.8 kg/m2, hypertension, and type II diabetes mellitus. Biopsy specimens obtained from skin lesions showed multiple dilated lymph nodes throughout the papillary and reticular dermis. Doppler ultrasonography and lymphoscintigraphy revealed lymph stasis and lymphedema, respectively. Based on clinical, histopathological, and imaging findings, the final diagnosis was ENV. She was initially treated with conservative approaches such as compression stockings and dietary intervention for a month. She went on a low-calorie diet (1350 kcal/day) consisting protein 50.7 g/day, fat 32.5 g/day, and carbohydrate 202.7 g/day, for a month. Owing to the hyperkeratotic lesions, she was given oral acitretin 0.3 mg/kg/day. The dosage was increased to 0.6 mg/kg/day after one month. The lesions were slightly flattened after 2 months of therapy. Acitretin was ceased. Early identification is crucial for preventing morbidity in patients with ENV. Management of ENV is challenging for physicians because of the lack of definitive treatment and poor prognosis. In the management of ENV, it is crucial to treat underlying causes, such as obesity. Acitretin induces loosening and thinning of the hyperkeratotic stratum corneum. Thus, it can result in improvement of cutaneous ENV lesions.