Pages

Friday, September 12, 2014

Elephantiasis nostras verrucosa: two cases


Chronic lymphedema (tissue swelling caused by impaired lymphatic flow) can be an extremely disfiguring and disabling condition. In the past, I've posted descriptions and photos of the disfigurement caused by lymphatic filarial disease, with cases from Haiti and from India. I've also described a case of scrotal lymphedema caused by chronic infections of the urinary tract. This post is about an associated dermatological condition - elephantiasis nostras verrucosa. Be warned - it's not pretty.

Elephantiasis nostras verrucosa is a rare, disfiguring complication of longstanding chronic lymphedema. Recurrent soft tissue infections worsen the condition. Disruption of lymphatic vessels due to trauma or surgery, venous stasis (sluggish blood flow in the veins), radiation, obstruction due to tumors, portal hypertension, obesity and congestive heart failure can all be the precipitating factors of this disease. The affected extremity swells, sometimes to huge proportions; the skin thickens, becomes lichenified and can become covered with knobbly growths. Treatment options include use of elastic bandages, pneumatic stockings, mechanical massage, oral retinoids and surgery. The goal is to restore function and to reduce physical disability. For refractory cases, microsurgical lymphovenous anastomosis can be performed. Amputation should be the last option.

The photo below shows the legs of a 63-year-old obese patient, with chronic lymphedema, tissue inflammation (note the reddened skin) and pronounced elephantiasis nostras verrucosa of the left lower extremity, where the swelling is much greater. 8 years earlier he had received treatment for colon cancer, consisting of surgery and radiotherapy to the lower abdomen. Chronic lymphedema developed after radiotherapy in both legs.




And this is a close-up shot of the left leg. Note the size of the swelling and the thickened, rough skin.




Berngard and Narayanan have described a rather more striking case - that of a 54-year-old man with morbid obesity and congestive heart failure, who sought medical attention, complaining of difficulty breathing at night, swollen legs and weight gain. Physical examination revealed a large abdominal pannus (a thick sheet of skin and fat tissue hanging down from the belly) with lichenified skin containing cobblestone-like nodules and papules with hyperpigmentation. This large, heavy "apron" completely encircled the patient’s genitals and left a fibrotic tract from which he urinated. Ick!



Huge pannus hanging down between an obese man's legs. 



A close-up of the knobbly, hyperpigmented growths on the abdomen.


The patient was treated for decompensated heart failure and was discharged home with outpatient follow-up to be evaluated for surgical removal of the pannus.


Literature:

Akturk HK, Gbadamosi-Akindele M. (2014) Elephantiasis nostras verrucosa. BMJ Case Rep.

Berngard SC, Narayanan V. (2011) Elephantiasis nostras verrucosa of the pannus. J Gen Intern Med 26(7): 810




No comments:

Post a Comment