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Elephantiasis nostras verrucosa on the legs and abdomen with morbid obesity in an Indian lady

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Elephantiasis nostras verrucosa on the legs and abdomen with morbid obesity in an Indian lady
Podila S Sarma MD1, Ashok Ghorpade MD MNAMS2
Dermatology Online Journal 14 (12): 20

1. Department of Medicine
2. Department of Dermatology, Venereology and Leprosy
J. L. N. Hospital & Research Centre, Bhilai Steel Plant, Bhilai, Chhattisgarh, India. psasarma@sify.com


Abstract

Elephantiasis nostras verrucosa (ENV) of the legs and abdomen in a morbidly obese woman with multiple medical problems is reported. The diagnosis was suggested by the classical clinical features and confirmed by histopathology. The patient succumbed due to her multisystem diseases. Elephantiasis nostras verrucosa involving the abdomen is uncommon and has been reported only five times in the past.



Figure 1
Figure 1. Morbidly obese woman with firm edematous plaques over the legs and abdominal skin

A 62-year-old chronically bedridden, morbidly obese woman (weighing 286 lbs.) with congestive heart failure, hypothyroidism, diabetic nephropathy, and chronic obstructive pulmonary disease was hospitalized in the Intensive Care Unit and subsequently succumbed to refractory cardiac failure. She had markedly edematous legs and abdominal wall. Her legs were fiery-red in appearance; legs and abdomen showed firm edematous plaques with a cobblestone appearance. Abdominal striae were prominent and edematous. (Fig. 1). Histopathological examination from the abdominal skin showed hyperkeratosis, parakeratosis, and acanthosis of the epidermis, with a scattered dermal inflammatory infiltrate and dilated lymphatic spaces in the papillary and reticular dermis.

Elephantiasis nostras verrucosa (ENV), is an unusual progressive cutaneous hypertrophy due to chronic lymphedema, and repeated inflammatory episodes. It usually manifests over the lower extremities as non-pitting edema with lichenification, hyperkeratotic papules, nodules, and verrucous, cobblestone-like plaques [1, 2, 3, 4]. Abdominal skin involvement in ENV is rare and has been reported in only five cases in the English literature [1, 5, 6, 7, 8]. The condition has also been reported rarely on the upper extremities, ears, face, scrotum, chest, genitalia, buttocks, and periorbital region [1, 2, 3, 6]. Histolopathology shows acanthosis and hyperkeratosis of the epidermis and dilated lymphatic spaces in the dermis.

Elephantiasis nostras verrucosa represents longstanding obstruction of the lymphatic drainage [7], leading eventually to grotesque enlargement of the chronically dependent and immobile part(s) of the body.

Chronic persistent lymphedema may lead to recurrent streptococcal lymphangitis, which can also be the predisposing factor [2, 3, 4]. The microorganisms may gain entry into the lymphatics through minor injuries, interdigital fissures, or tinea pedis. Recurrence is common due to the protein rich edema. In the previous reports of abdominal ENV, it was hypothesized that the massiveness of the panniculus caused increased interstitial and intravascular pressure predisposing the patients to chronic low-grade cellulitis and lymphangitis [5, 6]. Out of the five previously reported cases involving the abdomen, one showed improvement after weight loss and partial lipectomy and another one showed improvement with the use of topical tazarotene [1, 5].

The condition may also occur secondary to trauma, surgery, tumor-obstruction, radiation, portal hypertension, congestive heart failure and Kaposi sarcoma [2, 3, 8]. The differential diagnosis includes filariasis, pretibial myxedema, and stasis dermatitis. Obesity is a significant predisposing factor.

Treatment consists of antibiotics, compression stockings, topical keratolytics, pneumatic compression devices, oral retinoids, and surgical intervention. Management in advanced stages is unsatisfactory. Our patient was chronically bed-ridden and had several co-morbid factors including congestive cardiac failure, hypothyroidism, and morbid obesity with a massive apron of panniculus almost reaching up to the mid-thigh. It is important to recognize this rare condition in its initial stages in order to prevent debilitating deformities of the legs and other body parts [3].

References

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4. Saccoman S, Rifleman GT. Elephantiasis nostras verrucosa. J. Am. Podiatr. Med. Assoc. 1995; 85:265-267. [PubMed]

5. Chernosky ME, Derbes VJ. Elephantiasis nostras verrucosa of the abdominal wall. Arch. Dermatol. 1966; 94: 757-762. [PubMed]

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7. Hanna D, Cloutier R, Laporte R, Desgagne A. Abdominal elephantiasis: A case report. J. Cut. Med. Surg. 2004; 8: 229-232. [PubMed]

8. Sathya kumar S, Suh JS, Sharp VL, Polsky B. Elephantiasis nostras verrucosa secondary to Kaposi sarcoma: a rare case. AIDS. Read. 2008; 18: 81-82. [PubMed]

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