Case Letter

Elephantiasis Nostras Verrucosa Secondary to Scleroderma

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Practice Points

  • Scleroderma rarely may lead to elephantiasis nostras verrucosa (ENV) of the upper extremities.
  • Avoiding lymphostasis through compression and control of concomitant skin and soft tissue infections is important in the treatment and prevention of ENV.


 

References

To the Editor:

Elephantiasis nostras verrucosa (ENV) is a skin disorder caused by marked underlying lymphedema that leads to hyperkeratosis, papillomatosis, and verrucous growths on the epidermis.1 The pathophysiology of ENV relates to noninfectious lymphatic obstruction and lymphatic fibrosis secondary to venous stasis, malignancy, radiation therapy, or trauma.2 We present an unusual case of lymphedema and subsequent ENV limited to the arms and hands in a patient with scleroderma, an autoimmune fibrosing disorder.

A 54-year-old woman with a 5-year history of scleroderma presented to our dermatology clinic for treatment of progressive skin changes including pruritus, tightness, finger ulcerations, and pus exuding from papules on the dorsal arms and hands. She had been experiencing several systemic symptoms including dysphagia and lung involvement, necessitating oxygen therapy and a continuous positive airway pressure device for pulmonary arterial hypertension. A computed tomography scan of the lungs demonstrated an increase in ground-glass infiltrates in the right lower lobe and an air-fluid level in the esophagus. At the time of presentation, she was being treated with bosentan and sildenafil for pulmonary arterial hypertension, in addition to prednisone, venlafaxine, lansoprazole, metoclopramide, levothyroxine, temazepam, aspirin, and oxycodone. In the 2 years prior to presentation, she had been treated with intravenous cyclophosphamide once monthly for 6 months, adalimumab for 1 year, and 1 session of photodynamic therapy to the arms, all without benefit.

Physical examination showed cutaneous signs of scleroderma including marked sclerosis of the skin on the face, hands, V of the neck, proximal arms, and mid and proximal thighs. Excoriated papules with overlying crusting and pustulation were superimposed on the sclerotic skin of the arms (Figure 1).

Figure 1. Left forearm sclerosis with overlying papules and crust in a patient with elephantiasis nostras verrucosa.

A superinfection was diagnosed and treated with cephalexin 500 mg 4 times daily for 2 weeks; thereafter, mupirocin cream twice daily was used as needed. She was prescribed fexofenadine 180 mg twice daily and doxepin 20 mg at bedtime for pruritus.

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