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A 60-year-old man with chronic and recurrent bouts of lymphedema of the lower extremities had not been seen by a physician for several years. He presented with dozens of individual and coalescing dark pink-violet nodules on his lower extremities. The lesions were very pruritic and mildly painful.


What is your diagnosis?


Case of the Month 2012

Diagnosis:
Elephantiasis nostras verrucosa

Chronic lymphedema, lymphangitis, and obstruction of the major lymphatic channels of the skin can result in permanent hypertrophic nodular fibrosis. This end stage presentation has been called Elephantiasis nostras verrucosa (different form the elephantiasis tropica seen from lymphatic blockage from filarial worms).

At this point it is extremely difficult to treat.

Treatment:

  1. Primary care physician for the management of the underlying causes of the lymphedema (see Cutis article, below).
  2. For symptomatic relief and to control additional development and progression knee-high, compression stockings that are custom fit with the pressure of 30 mmHg Mercury or higher can be beneficial to ship they should be worn as much as possible throughout the day with an application of triamcinolone 0.1% cream applied once daily. Although not perfect, many patients report a significant and satisfactory improvement of both the symptoms and the condition with this treatment protocol.

 

Reference:

Cutis. 1998 Aug;62(2):77-80.
Elephantiasis nostras verrucosa.
Schissel DJ, Hivnor C, Elston DM.

Source:
Department of Dermatology, Brooke Army Medical Center, Fort Sam Houston, Texas 78234-6200, USA.

Abstract:
Elephantiasis nostras verrucosa is a rare, chronic, deforming disorder characterized by hyperkeratosis and papillomatosis of the epidermis with underlying woody fibrosis of the dermis and subcutaneous tissue. Chronic lymphedema, either congenital or secondary to infection, surgery, radiation, neoplastic obstruction, obesity, portal hypertension, or chronic congestive heart failure, plays a pivotal role in the pathogenesis. Without appropriate intervention, the slowly progressive cutaneous changes will culminate in massive and grotesque enlargement of the affected body region. In the following case report, the natural history and the management of elephantiasis nostras are discussed.

For full article see:
http://www.ncbi.nlm.nih.gov/pubmed/9714902



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