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Elephantiasis Nostras Verrucosa on the buttocks and sacrum of two immobile men

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Elephantiasis Nostras Verrucosa on the buttocks and sacrum of two immobile men
Hedy G Setyadi1 MD, Megan Reif Iacco2 BA, Tor A Shwayder1 MD, Adrian Ormsby3 MD
Dermatology Online Journal 17 (2): 8

1. Department of Dermatology, Henry Ford Health System, Detroit, Michigan
2. University of Michigan Medical School, University of Michigan Health System, Ann Arbor, Michigan
3. Department of Pathology, Henry Ford Health System, Detroit, Michigan


Abstract

Though typically involving the lower extremities, elephantiasis nostras verrucosa (ENV) can occur in any area affected by lymphedema. Here we report two cases of ENV: one is a biopsy-proven case and the other is a clinically diagnosed case. Both occurred on the buttocks and sacrum of immobile, morbidly obese men who were persistently in the supine or seated position. Whereas classic ENV is not uncommon, this striking presentation on these unusual areas is quite rare.



Case 1


Figure 1Figure 2

A 33-year-old man with a history of morbid obesity, Down syndrome, hypertension, hypothyroidism, and deep venous thrombosis presented with a 6-year history of extensive multiple firm exophytic, coalescing, cobblestone-like erythematous papules and nodules arising from large erythematous lichenified plaques overlying the buttocks and sacrum (Figure 1). Physical exam also revealed scrotal edema and contraction of both feet resulting in immobility.

Histopathology of one of the nodules revealed a polypoid lesion with epidermal hyperplasia, ulceration, dermal edema, fibrosis, dilated lymphatic vessels, and chronic inflammation, consistent with ENV (Figure 2). The patient was managed with frequent repositioning and zinc oxide paste to the affected area. His family declined any further intervention.


Case 2


Figure 3

A 45-year-old man with history of morbid obesity, muscular dystrophy resulting in immobility since his teens, severe lymphedema, multiple decubitus ulcers, hypertension, and hidradenitis suppurativa, presented with hyperkeratotic, verrucous, cobblestone-appearing nodules on the lower extremities consistent with the classic clinical presentation of ENV. Physical examination was also remarkable for multiple firm exophytic, coalescing, cobblestone-like erythematous-to-hyperpigmented papules and nodules arising from large hyperpigmented lichenified plaques over the sacrum and superior buttocks (Figure 3).These lesions were clinically diagnosed as ENV. His scrotum was noted to be erythematous with generalized edema. The patient declined biopsy of the sacral/buttock lesions. Management consisted of leg elevation and diuresis as well as local wound care.


Discussion

Although typically involving the lower extremities, elephantiasis nostras verrucosa (ENV) can occur in any area affected by chronic lymphedema and has been reported on the abdomen, upper extremities, ears, face, and genitals [1]. Clinically, ENV is characterized by gross enlargement of the affected region with a verrucous, papillomatous, cobblestone-like appearance. Histologic changes include pseudoepitheliomatous hyperplasia, dermal fibrosis, and dilated lymphatic vessels [2, 3]. The exact mechanism is unknown, but the purported pathogenesis hinges upon the presence of lymphostasis.

There are many causes of secondary lymphedema, including obesity, infectious etiologies, neoplasia, trauma, congestive heart failure (CHF), portal hypertension, and radiation, but the rate of obesity is rapidly increasing in the United States [3]. Excess adipose tissue may result in impaired lymphatic flow, precipitating the accumulation of interstitial fluid and initiating a cascade of fibrosis, chronic inflammation, and susceptibility to infection [4]. Our two patients’ morbid obesity, coupled with their immobility, may have been the inciting factors for development of ENV of the buttocks and sacrum. The differential diagnosis for similar lesions in the anogenital region would include verrucous carcinoma of the Buschke-Lowenstein subtype.

Treatment of ENV remains difficult. Initial management is aimed at improving lymphedema. Other therapies including antibiotic prophylaxis, oral and topical retinoids, topical keratolytics, and surgical interventions such as debridement, lymphaticovenular anastamosis, and amputation, have been employed with varying success [1, 5].

References

1. Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol 2008;9(3):141-6. [PubMed]

2. Yang YS, Ahn JJ, Haw S, Shin MK, Haw CR. A case of elephantiasis nostras verrucosa. Ann Dermatol. 2009 Aug;21(3):326-9. [PubMed]

3. Baird D, Bode D, Akers T, Deyoung Z. Elephantiasis nostras verrucosa (ENV): a complication of congestive heart failure and obesity. J Am Board Fam Med. 2010 May-Jun;23(3):413-7. [PubMed]

4. Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007 Jun;56(6):901-916. [PubMed]

5. Motegi S, Tamura A, Okada E, Nagai Y, Ishikawa O. Successful treatment with lymphaticovenular anastamosis for secondary skin lesions of chronic lymphedema. Dermatology. 2007;215(2):147-151. [PubMed]

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