Case Report

Elephantiasis Nostras Verrucosa: Interest in Acting Early!

Imane Kacimi Alaoui*, Hanane Baybay, Zakia Douhi, Meryem Soughi, Sara Elloudi and Fatima Zahra Mernissi

Department of Dermatology, University Hospital Hassan II, Morocco

Received Date: 21/02/2023; Published Date: 18/04/2023

*Corresponding author: Dr. Imane Kacimi Alaoui, Department of Dermatology, University Hospital Hassan II, Fes, Morocco

DOI: 10.46998/IJCMCR.2023.25.000619

Abstract

Elephantiasis nostras verrucosa is a complication of chronic lymphedema with progressive dermal fibrosis, epidermal changes, hyperkeratosis, and papillomatosis, resulting in a classic cobblestone appearance. We report a new observation of ENV in a polypathological patient.

Introduction

Elephantiasis nostras verrucosa (ENV) is a rare disease marked by chronic non-filarial lymphedema resulting in lymphatic obstruction, leading to skin hypertrophy with disabling complications [1].

Observations

A 75-year-old female patient with a history of recurrent bilateral erysipelas complicated by lymphedema, who suffered from congestive heart failure, diabetic nephropathy, and obesity, and also complained of worsening dyspnea on exertion. His surgical, travel, and family history were unremarkable.

On physical examination, her lower extremities were enlarged with papillomatous and verrucous lesions (Figure 1, 2). Kaposi-Stemmer sign was present.

Figure 1: Two inferior limbs increased in size.

Figure 2: Papillomatous and verrucous appearance.

Discussion

Elephantiasis Nostras Verrucosa (ENV) was initially defined as a condition resulting from lymphatic blockage caused by neoplasms, trauma, radiation therapy, congestive heart failure, obesity, hypothyroidism, chronic venous stasis, and filarial infection [2,3].

The lower extremities are the most common site of ENV. Initially, it presents as mild, persistent edema. Then, the skin loses its elasticity becoming hypertrophic, warty and scaly.

Complications may be infectious (mycotic intertrigo or erysipelas), dysimmune or neoplastic (angiosarcoma) [4].

It must be differentiated from pretibial myxedema, filariasis, lipedema, chromoblastomycosis, lipodermatosclerosis and venous stasis dermatitis [4,5].

In the management of ENV, it is crucial to treat the underlying causes. Compression with elastic bandaging is considered an effective treatment. Systemic diuretics and antibiotics may be necessary to reduce edema and control infection.

The expected survival of a patient with ENV is based on the severity of lymphedema, underlying diseases, and other contributing factors. Early diagnosis and early intervention in the vicious cycle will result in better outcomes [3].

Conclusion

Therefore, it is imperative to recognize this rare disease in its early stages and to control infection and edema as soon as possible to prevent debilitating deformities.

Consent: The examination of the patient was conducted according to the Declaration of Helsinki principles.
Conflicts of interest: The authors do not declare any conflict of interest

References

  1. Fife CE, Carter MJ. Lymphedema in the morbidly obese patient: Unique challenges in a unique population. Ostomy Wound Manag. 2008; 54: 44–56.
  2. Sinha P, Tripathy DM, Radhakrishnan S, Verghese B, Sunita BS. Tuberculosis verrucosa cut is presenting with unilateral elephantiasis nostras verrucosa of the left lower limb: A rare entity. J. Mycobacteriol, 2019; 8: 202–204.
  3. Castellani A. Researches on elephantiasis nostras and elephantiasis tropica with special regard to their initial stage of recurring lymphangitis (lymphangitis recurrens elephantogenica). Supplementary note. Trop. Med. Hyg, 1969; 72: 214–215.
  4. Damstra RJ, Dickinson-Blok JL, Voesten HG. Shaving Technique and Compression Therapy for Elephantiasis Nostras Verrucosa (Lymphostatic Verrucosis) of Forefeet and Toes in End-Stage Primary Lymphedema: A 5 Year Follow-Up Study in 28 Patients and a Review of the Literature. J Clin Med, 2020; 9(10): 3139. doi: 10.3390/jcm9103139. PMID: 32998425; PMCID: PMC7601471.
  5. Mugarza Hernández MD, Iglesias Cano O, Ayllón Blanco H. Elefantiasis verrucosa nostra [Elephantiasis nostra verrucosa]. Aten Primaria, 2019; 51(8): 521-522. Spanish. doi: 10.1016/j.aprim.2018.12.005. Epub 2019 Feb 15. PMID: 30777387; PMCID: PMC6837003.
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