• Users Online: 397
  • Home
  • Print this page
  • Email this page

 Table of Contents  
Year : 2019  |  Volume : 8  |  Issue : 2  |  Page : 202-204

Tuberculosis verrucosa cutis presenting with unilateral elephantiasis nostras verrucosa of the left lower limb: A rare entity

Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India

Date of Web Publication14-Jun-2019

Correspondence Address:
Preema Sinha
Department of Dermatology, Armed Forces Medical College, Pune - 411 040, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijmy.ijmy_38_19

Rights and Permissions

Elephantiasis nostras verrucosa is used to describe lymphedema of the lower limbs caused by a nonfilarial etiology. Infective etiologies include bacterial and fungal infections along with chronic venous stasis, postirradiation, congestive heart failure, obesity, and malignancies, all causes leading to lymphatic obstruction and edema. We herein describe a case of a 70-year-old male who presented with diffuse nonpitting edema of the entire left lower limb with cobblestoning of the surface with a positive Kaposi–Stemmer sign and presence of a verrucous plaque over the left ankle. Histopathology of the verrucous plaque revealed it to be tuberculosis verrucosa cutis (TVC). We report this rare presentation of TVC as a cause of unilateral lymphedema.

Keywords: Lymphedema, rare, tuberculosis verrucosa cutis

How to cite this article:
Sinha P, Tripathy DM, Radhakrishnan S, Verghese B, Sunita B S. Tuberculosis verrucosa cutis presenting with unilateral elephantiasis nostras verrucosa of the left lower limb: A rare entity. Int J Mycobacteriol 2019;8:202-4

How to cite this URL:
Sinha P, Tripathy DM, Radhakrishnan S, Verghese B, Sunita B S. Tuberculosis verrucosa cutis presenting with unilateral elephantiasis nostras verrucosa of the left lower limb: A rare entity. Int J Mycobacteriol [serial online] 2019 [cited 2023 Jan 30];8:202-4. Available from: https://www.ijmyco.org/text.asp?2019/8/2/202/260373

  Introduction Top

Elephantiasis is described as disfiguring enlargement of a body part, most commonly lower limbs as a result of edema and fibrosis of skin. Chronic lymphedema and its accompanying sequelae are central to the pathogenesis of elephantiasis.[1],[2] Lymphedema can be primary or secondary. Congenital hypoplasia or agenesis as seen in Milroy's disease results in primary lymphedema while infections, malignancies, chronic venous stasis, post-radiation, obesity, and congestive heart failure are among the common causes of secondary lymphedema.[3]

Lymphedema of the external genitalia mostly affects the vulva with lymphogranuloma venereum being the most common cause followed by filariasis and tuberculosis. In endemic countries, lymphatic filariasis is the most common cause of elephantiasis and is responsible for profound physical disability.[4] Herein, we describe one such case of elephantiasis of lower limb where the etiology was cutaneous tuberculosis which is a rare presentation.

  Case Report Top

A 70-year-old male presented with complaints of a raised dark-colored lesion over the dorsum of the left foot associated with gradually progressive generalized swelling of the left lower limb for the past 6 months. The generalized swelling had progressed from the foot distally up till the thighs. There was no history of discharge or bleeding from the raised growth. There was a history of weight loss of 5 kg over the past 3 months without any associated loss of appetite, fever, night sweats, cough, or any other systemic symptoms. Past, family, and personal histories were not contributory.

The general examination revealed multiple nontender lymphadenopathies over the axillary and inguinal region with the largest measuring 2 cm × 2 cm in the left inguinal region. Dermatological examination revealed a 3 cm × 3 cm verrucous growth over the dorsum of left foot [Figure 1]. There was diffuse nonpitting edema involving the entire left lower limb up to the thighs [Figure 2]. Kaposi–Stemmer sign was positive.
Figure 1: Approximately 3 cm × 3 cm size verrucous growth over the dorsum of left foot

Click here to view
Figure 2: Diffuse nonpitting edema involving the entire left lower limb

Click here to view

Investigations revealed normal hematological and biochemical parameters. Peripheral blood smear for microfilariae was negative. Radiological investigations including contrast-enhanced computerized tomography of the chest and abdomen showed no other evidence of infection or malignancy, and Doppler studies were also normal. Fine-needle aspiration cytology of the left inguinal lymph node was suggestive of necrotizing lymphadenitis with granulomatous caseous necrosis but was negative for acid-fast bacilli. A skin biopsy from the verrucous plaque revealed pseudoepitheliomatous hyperplasia with fibrosis and granulomas in dermis composed of neutrophils, lymphocytes, and plasma cells [Figure 3] and [Figure 4]. Multinucleate giant cells of Langhans type and foreign body type were also noted. Periodic acid–Schiff and Grocott stains to rule out fungal infections were negative. On the basis of the clinical and histopathological picture, a diagnosis of tuberculosis verrucosa cutis (TVC) was made.
Figure 3: Epidermis shows pseudoepitheliomatous hyperplasia with multiple foci of fibrosis evident in the dermis (H and E, ×100)

Click here to view
Figure 4: Dermis shows large areas of necrosis and mixed inflammatory infiltrate predominantly composed of neutrophils, lymphocytes, eosinophils, and plasma cells with karyorrhectic debris. Epithelioid cell granulomas, multinucleated foreign body giant cells, and Langhans giant cells are also noted (H and E, ×400)

Click here to view

The patient was started on antitubercular therapy. He was also advised pneumatic compression stockings for the lymphedema along with regular limb massage and physiotherapy. He has shown a good response to therapy with gradual improvement in the verrucous plaque as well as regression of the lymphedema. The patient is under regular follow-up.

  Discussion Top

Elephantiasis nostras verrucosa (ENV) is a unique entity which is an accentuated form of chronic lymphedema of nonfilarial etiology. Castellani in 1969 had divided elephantiasis into four groups namely elephantiasis tropica due to filariasis; elephantiasis nostras due to bacterial infections; elephantiasis symptomatica due to either fungal, tubercular, or malignancies; and elephantiasis congenita due to inherited diseases such as Milroy's disease.[5]

Initially described as lymphangitis recurrens elephantogenica due to recurrent bacterial infection of the lymphatics, ENV, as per recent literature also encompasses tubercular, fungal, and neoplastic etiologies. Term “nostras” was added in 1934 to delineate the disorder from filarial elephantiasis of tropical regions.[4]

The exact pathogenesis of ENV is yet to be deciphered. It is postulated that recurrent lymphangitis caused by the above-mentioned triggers results in damaged lymphatics leading to extrusion of protein-rich fluid into the dermis and subcutis.[3] A cascade of inflammatory reaction begins that ultimately results in edema, initially pitting, and gradually becomes nonpitting with extensive fibrosis. Weakened local immunity also predisposes to further microbial invasion, and the vicious cycle continues.[1],[3]

Recurrent streptococcal lymphangitis is generally the most common cause for such debilitating lymphedema.[6] Although cutaneous tuberculosis has been associated with lymphangitis, it leading to chronic lymphedema and ENV has hardly been mentioned in literature.[4],[7] TVC is an exogenous form of cutaneous tuberculosis that presents as a verrucous plaque or nodule at sites of trauma most commonly lower limbs. It is a slowly progressing form of tuberculosis that responds very well to antitubercular therapy. Very rarely, if not treated, it can result in secondary lymphedema and subsequently ENV.[8]

ENV usually begins as pitting edema of distal limb giving a mossy appearance and progresses proximally to involve the entire limb in a few years. The edema becomes nonpitting due to profound fibrosis and results in a woody hard consistency and cobblestoning on the surface.[1],[3] Kaposi–Stemmer sign which is the inability to pinch the skin on the dorsal aspect of the second toe is a very specific sign of chronic lymphedema which was also positive in our case.[3]

Histopathological examination usually reveals pseudoepitheliomatous hyperplasia along with dilatation of the lymphatic space and extensive fibrosis but not all findings are always present. Lymphangiography, lymphoscintigraphy, and magnetic resonance imaging give further cues toward ENV and help in ruling out malignancies. All causes have to be ruled out including blood for microfilariae.[1],[3]

The management of ENV is broadly two folds. We first treat the cause leading to ENV which in our case was tuberculosis verrucosa cutis, hence antitubercular treatment was started. Second, the management of ENV per se involving general measures such as limb elevation, limb physiotherapy, and pneumatic compression stockings alleviates symptoms to some extent. Diuretics reduce edema and have been used frequently along with systemic retinoids which have also proven to be beneficial in chronic lymphedema. In extremely rare scenarios, surgical debridement and even amputation may be necessary for recalcitrant lymphedema.[3],[4]

  Conclusion Top

TVC as a cause of ENV hardly finds a mention in literature making it a very unique clinical presentation. The aim of management is to block the cascading event that triggers chronic lymphedema and prevent severely debilitating physical disability.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Liaw FY, Huang CF, Wu YC, Wu BY. Elephantiasis nostras Verrucosa: Swelling with verrucose appearance of lower limbs. Can Fam Physician 2012;58:e551-3.  Back to cited text no. 1
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;23:413-7.  Back to cited text no. 2
Yang YS, Ahn JJ, Haw S, Shin MK, Haw CR. A case of elephantiasis nostras Verrucosa. Ann Dermatol 2009;21:326-9.  Back to cited text no. 3
Dreyer G, Norões J, Figueredo-Silva J, Piessens WF. Pathogenesis of lymphatic disease in bancroftian filariasis: A clinical perspective. Parasitol Today 2000;16:544-8.  Back to cited text no. 4
Dean SM, Zirwas MJ, Horst AV. Elephantiasis nostras Verrucosa: An institutional analysis of 21 cases. J Am Acad Dermatol 2011;64:1104-10.  Back to cited text no. 5
Turhan E, Ege A, Keser S, Bayar A. Elephantiasis nostras Verrucosa complicated with chronic tibial osteomyelitis. Arch Orthop Trauma Surg 2008;128:1183-6.  Back to cited text no. 6
Sequeira JH. Tuberculous lymphangitis of skin. Proc R Soc Med 1923;16:101.  Back to cited text no. 7
Masellis P, Gasparini G, Caputo R, Alessi E. Tuberculosis Verrucosa cutis which remained undiagnosed for forty-three years. Dermatology 1995;191:145-8.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded245    
    Comments [Add]    

Recommend this journal