Penoscrotal Elephantiasis nostras verrucosa: A case report and literature review

BACKGROUND: Elephantiasis Nostras Verrucosa (ENV) is a rare clinical condition associated with chronic non-filarial lymphedema caused by bacterial or non-infectious lymphatic obstruction. CASE DESCRIPTION: We reported an unusual case of isolated peno-scrotal ENV of a 67-year-old man with a history of Gastrointestinal Stromal Tumors (GIST) of the stomach. Our patient presented for progressive painless lymphedema of the penis and scrotum since 8 years ago before the diagnosis of GIST tumors, associated with skin changes consistent of superimposed hyperkeratotic papulonodules with a verrucose or cobblestone-like appearance. The patient refused surgical excision. He was treated with oral Acitretin 30 mg/day for 4 weeks where a minimal improvement was noted. Acitretin was stopped due to an increase in liver enzymes. Conservative treatment was applied to decrease lymphostasis. DISCUSSION: ENV is characterized by lymphedema and skin changes consisting of hyperkeratotic, verrucous and papillomatous lesions. It is most commonly caused by bacterial infection, trauma, neoplasia and obesity. The diagnosis of ENV is achieved clinically by history and typical skin changes. Imagery and skin biopsy are used to differentiate ENV from other diseases. Management of ENV remains challenging. Strategies to reduce lymph stasis include lymphatic massages, compressive dressings are often inadequate. Surgical debridement may be considered in cases where there is no improvement depsite medical therapy. Oral retinoids can be used if tolerated with variable results. CONCLUSION: ENV is a rare disorder that results from chronic obstructive lymphedema. There is no standard therapy but a variety of medical and surgical treatment options have been reported.