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Lymphedema Skin Plaque and Nodules

Postby patoco » Sat Jun 10, 2006 4:22 pm

Lymphedema Skin Plaque and Nodules

Elephantiasis nostras verrucosa

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Related Terms:

Armchair legs, Chronic recurrent streptoccocal erysipelas, Deckchair legs, Elephantiasis nostras, Lymphoedematous hyperkeratosis, Lymphoedematous papillomatosis, Mossy foot, Wheelchair legs

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Often in late stage and long term lymphedema, patients begin to experience a dramatic change in the appearance, texture and condition of the skin. While the underlying subcutaneous tissue becomes woody, the skin becomes increasing thick, and acquires a "cobblestone" appearance (hyperkeratosis)with small flesh or even reddish colored nodules popping up (papillomatosis).

Medically, this condition is reffered to as elephantiasis nostras verrucosa.

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Elephantiasis nostras verrucosa

Schissel DJ, Hivnor C, Elston DM.

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

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.

Publication Types:
Case Reports

http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Abstract

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Elephantiasis nostras verrucosa

Woody Edema of the Legs

A 69-year-old man presented with severe bilateral lower extremity woody edema, pebbly hyperkeratotic plaques, and a fetid odor (see accompanying figure). Crusts were easily dislodged and left weeping erosions. This condition had progressed over many years of chronic lymphedema and recurrent skin infections.

Discussion

The answer is B: elephantiasis nostras. It is a disorder in which chronic lymphedema and recurrent skin infection result in severely deformed fibrotic enlargement of the affected extremity.1-4 The epidermis becomes cobblestoned with a loosely adherent crust, which is often foul smelling and may be colonized with multiple bacteria and fungi.2-4

The differential diagnosis includes venous stasis dermatitis, pretibial myxedema, filariasis, and ichthyosis. Venous stasis dermatitis, caused by chronic venous insufficiency, is characterized by pitting edema and hemosiderin-stained, tight skin in which minor trauma causes ulceration. Pretibial myxedema, associated with Graves' disease, is caused by intradermal mucin accumulation. The usual manifestation is bilateral pretibial flesh-colored nodules or plaques. Filariasis can cause localized lymphedema. Blockage of lymph channels by filarial worms leads to edema most commonly affecting the legs and genital area. If filariasis is suspected on the basis of a travel history to an endemic tropical area, it can be diagnosed by serologic studies or by finding filarial worms in body fluids. Ichthyosis is a general term for a variety of genetic and acquired disorders that lead to development of thickened hyperkeratotic scales on the skin. Unlike elephantiasis nostras, there is no history of recurrent infection or chronic edema leading to the formation of the hyperkeratotic skin changes.

Lymphedema, regardless of etiology, is a predisposition for the development of elephantiasis nostras. However, it is the recurrent infections that cause the most damage. Repeated episodes of inflammation lead to fibrosis of the dermis and lymph channels that ultimately results in elephantiasis nostras.5-7

Treatment of elephantiasis nostras aims at prevention of recurrent infection and edema. Compressive stockings are used to decrease edema. In severe cases, pneumatic compression devices may be useful.6 Prolonged courses of antibiotics may be needed to clear deep-seated infections. Topical keratolytics and oral retinoids have been used to treat hyperkeratotic plaques, but chronic use is sometimes required to maintain the results and is not risk free.7 Recalcitrant cases may benefit from surgical intervention such as lymphovenous anastomosis but ultimately may require amputation. Early treatment of infection to eradicate bacteria and preserve the lymphatic drainage of dependent extremities is essential.

QUENBY L. ERICKSON, CAPT, USAF, MC
TODD KOBAYASHI, MAJ, USAF, MC, FS
PAULA S. VOGEL, LTC, MC, USA
Wilford Hall Medical Center
2200 Berquist Dr.
Lackland AFB, TX 78236-5300

http://www.aafp.org/afp/20030201/photo.html

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Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy.

Zouboulis CC, Biczo S, Gollnick H, Reupke HJ, Rinck G, Szabo M, Fekete J, Orfanos CE.

Department of Dermatology, University Medical Center Steglitz, Free University of Berlin, Germany.

Elephantiasis nostras verrucosa is characterized by chronic secondary, non-filarial lymphoedema due to recurrent lymphangitis, dermal fibrosis, and epidermal changes consisting of hyperkeratotic, verrucous and papillomatous lesions. Histologically, there is pseudoepitheliomatous hyperplasia. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes. In this study, rapid disappearance of the hyperkeratotic and verrucous lesions, remarkable flattening of the papillomatous nodules and improvement of lymphoedema occurred in three obese patients treated with etretinate in an initial dose of 0.6-0.75 mg/kg/day for 4-6 weeks. Monitoring of plasma concentrations of etretinate, acitretin and 13-cis-acitretin by HPLC revealed sufficient short-time absorption (4 h) and bioavailability of the drug (30 days; two out of three patients). Long-term maintenance therapy in one patient produced a remarkable improvement in the lymphoedema; another patient relapsed after discontinuation of the etretinate and responded again after this was reintroduced. In the third patient treatment was withdrawn because of an increase in triglycerides, but improvement persisted 6 months later. The clinical side-effects of oral retinoid therapy were moderate and well tolerated.

Publication Types:
Case Reports

PMID: 1419764 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... =iconabstr

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Nostras verrucosa successfully treated by surgical debridement.

Iwao F, Sato-Matsumura KC, Sawamura D, Shimizu H.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

BACKGROUND: Elephantiasis nostras verrucosa represents a rare group of cutaneous changes comprising dermal fibrosis, hyperkeratotic, verrucous, and papillomatous lesions after chronic secondary, nonfilarial lymphedema. There is no standard treatment for this rare cutaneous manifestation. OBJECTIVE: This article describes debridement that is helpful when performed in addition to physiotherapy. METHODS: We present a patient who was treated by shaving the verrucous area with a blade of a freehand knife and by subsequent abrading of the mossy area using a motor-powered grinder. RESULTS: Reepithelization was completed in 2 weeks. A compression stocking was used to treat the lymphedema. Ten months after the operation, we saw no signs of disease recurrence. The result was also aesthetically satisfactory. CONCLUSIONS: Surgery in such case may not always be the best treatment because it does not treat the cause of disease but only treats the cutaneous symptoms. Nevertheless, debridement is a rapid and an aesthetically acceptable form of treatment.

Publication Types:
Case Reports

PMID: 15171776 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/quer ... =iconabstr

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Elephantiasis nostras verrucosa

Mario Vaccaro, MD, PhD , Francesco Borgia, MD, Fabrizio Guarneri, MD and Serafinella P. Cannavò, MD

A 62-year-old white man was referred to our department in May 1998 because of the progressive development of chronic lymphedema on the lower extremities; since the age of 58 years, he had noted recurrent swelling with bilateral edema involving, at first, the dorsum of the feet and, later, both the front and the back of the legs, leading to the advancing deformation of the entire extremities.

His medical history was significant for congestive heart failure and severe obesity; anamnestic investigation was negative for filariasis and familial Milroy's disease.

Physical examination revealed a man in poor health with diffuse thickening of both feet and legs, accompanied by profound, nonpitting edema, generalized lichenification, and cobblestone-like skin surface. Severely malodorous verrucous plaques were present on the calves; ulcerative lesions were also noted on the medial malleolus of the left extremity

( Fig. 1). The toes of both feet showed marked pachyonychia and signs of onycogryphosis.

Posterior tibial and dorsal pedis pulses were not palpable bilaterally. Laboratory tests revealed slightly elevated serum levels of sugar, triglycerides, and cholesterol. Repeated cultures from lesional areas showed Trichophyton rubrum, Candida albicans, and -hemolytic Streptococcus group A.

A surgical biopsy specimen obtained from the edge of the ulcer showed hyperkeratosis with parakeratosis and acanthosis of the epidermis; diffuse edema and multiple dilated lymphatic spaces throughout the papillary and reticular dermis were observed. There was no sign of neoplastic change ( Fig. 2).

The patient was given oral itraconazole 200 mg/day and amoxicillin plus clavunalate 1 g twice daily, but treatment was stopped because of the increased cardiac decompensation and subsequent death.

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Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene

http://www.findarticles.com/p/articles/ ... _n12417040

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Elephantiasis nostras verrucosa

Diagnostic Images

http://www.aafp.org/afp/20030201/photo.html

http://images.google.com/images?q=eleph ... rch+Images
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