Pages 209 - 214 Volume 91, Issue 2
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Elephantiasis nostras verrucosa secondary to leprous lymphadenopathy; a case report

Leprosy has earned the status of ‘great imitator’ considering its ability to present in wide array of manifestations. Typical neuro-dermatological features are easy to recognize but uncommon presentations frequently delay the diagnosis. Here we report a 60-years-old lady presenting with elephantiasis nostras verrucosa secondary to lepromatous leprosy. In the post elimination era, it is important to have a high index of suspicion to prevent delay in diagnosis.

Cite this article
Rashmi Jindal, Nadia Shirazi, Payal Chauhan, Robin Chugh;
Elephantiasis nostras verrucosa secondary to leprous lymphadenopathy; a case report; Leprosy Review; 2020; 91; 2; 209-214; DOI: 10.47276/lr.91.2.209
LEPROSY
Leprosy Review
0305-7518
British Leprosy Relief Association
Colchester, UK
Introduction
Elephantiasis nostras verrucosa (ENV) is non-filarial lymphedema resulting in massive swelling, skin thickening and dermal as well as sub-dermal fibrosis. Primary differentiation is from elephantiasis tropica with underlying filarial etiology. The precursor of ENV is secondary lymphedema causing an acquired decrease in lymph flow. This can occur due to recurrent cellulitis, deep venous thrombosis, post-operatively (following abdominal surgery), obesity, renal insufficiency and congestive cardiac failure.1 Here we report a patient with elephantiasis nostras verrucosa of the leg, secondary to inguinal lymph node involvement by leprosy. Clinically perceptible lymph node involvement is uncommon in leprosy except during reactions.2 There were no clinical features to suggest reaction in the reported patient and the diagnosis of leprosy was established on lymph node biopsy. In the post-elimination era, widespread disease involving lymph nodes is uncommon and thus warrants attention to help in modification of future policies.
Figure 1.
Non-pitting edema of left leg with thickening and cobble-stoning of overlying skin and areas of lichenification superimposed by superficial ulcers.
Case report
A 60-years-old lady presented with progressively increasing swelling of the left leg extending to the mid-thigh, associated with thickening of the skin and superficial weeping ulcers over the ankle and dorsum of the foot for the previous one year. There was no family history of lymphedema. There was no history of recurrent cellulitis, trauma, varicose veins or any surgical intervention in the past. On general physical examination she had pallor and bilateral superficial inguinal lymphadenopathy which were multiple, firm, discrete and non-tender ranging from 1 × 1 cm to 2 × 2 cm in size. Her body mass index was 23.2. On cutaneous examination she had multiple smooth, hypo-pigmented macules distributed symmetrically over the back, chest and extremities. Sensation in these macules as well as on the extremities was intact. Both ulnar nerves, lateral cutaneous branches of the radius and common peroneal nerves were thickened and non-tender. There was non-pitting edema of left leg up to the mid-thigh with thickening and cobble-stoning of overlying skin and areas of lichenification, superimposed with superficial ulcers covered with yellowish slough over the ankle and dorsum of the foot near the base of fourth and fifth toes (Figure 1). Her hemoglobin was 8.66 gm/dl and other routine tests, liver function test, kidney function test and urine routine analysis were normal. Diethylcabamazine provocation test for microfilaria was negative. However, specific tests for filariasis (Filariasis test strip, Biplex Wb123/Ov16 and PCR) were not available. The patient lived in Dehradun district of Uttarakhand (India), which is a region non-endemic for filariasis. Slit skin smears done from both ears and stained with modified Ziehl Neelsen stain were positive for acid-fast bacilli (Mycobacterium leprae) with a bacillary index of 3+ and a morphological index of 10–15%. A skin biopsy sent from the leg showed hyperkeratosis, acanthosis and papillomatosis. There were a few ill-defined granulomas in the dermis surrounded by lymphocytes (Figure 2). A lymph node biopsy revealed a well-encapsulated lymph node extensively infiltrated by foamy macrophages, which on fite stain were positive for acid-fast bacilli (Figure 3). Doppler ultrasound of the left leg showed normal venous drainage. Due to financial constrains lymphoscintigraphy and magnetic resonance imaging of leg could not be performed. The patient was thus diagnosed as lepromatous leprosy with secondary elephantiasis nostras verrucosa. She was started on multidrug therapy (dapsone, clofazimine and rifampicin). For the ulcers over the ankle and leg, a seven-day course of oral amoxicillin and topical mupirocin was given. During follow-up she was advised to wear compression stockings and lifestyle modifications were suggested to improve lymphatic drainage of the leg.
Figure 2.
Foamy macrophage granulomas in dermis surrounded by lymphocytes 10X (Inset 40X).
Figure 3.
Well-encapsulated lymph node extensively infiltrated by foamy macrophages 4X and inset showing acid fast bacilli on fite stain in oil immersion.
Discussion
Lymphedema is considered the hallmark of elephantiasis and may be primary (congenital) or secondary (acquired). Secondary lymphedema can result from systemic or regional etiologies (Figure 4). Due to lymphatic obstruction, protein rich fluid seeps into the interstitial spaces inducing a chronic inflammatory response and decreasing local immunity.1 This predisposes the tissues to more infection and further fibrosis, setting up a vicious cycle. The end result is massive swelling and skin thickening, associated with dermal and sub-dermal fibrosis, termed elephantiasis. On the basis of the underlying etiology, five common types of elephantiasis have been described (Table 1).3 In 1934 Aldo Castellani first used the term elephantiasis nostras to distinguish it from its tropical counterpart caused by Wuchereria species commonly known as filariasis.3 Recurrent soft tissue infections in predisposed individuals are the most reported cause of elephantiasis nostras. These infections are predominantly staphylococcal, while Mycobacterium leprae is rarely reported as the cause of elephantiasis nostras.
Figure 4.
Causes of lymphedema.
Table 1
Types of elephantiasis on the basis of etiology
TypeEtiology
1NostrasNon-filarial
2TropicaFilarial
3PodoconiosisObstruction of lymphatic vessels by inorganic material from the soil
4GenitalLymphogranuloma venereum associated
5Proteus syndromeGenetic disorder (elephant man)
In the past, lymph node enlargement was commonly seen in patients with leprosy. A report of 105 leprosy patients from India in 1983 states that 100% of their patients with mid-borderline leprosy, borderline-lepromatous leprosy and lepromatous leprosy had enlarged lymph nodes.4 Another report states that involvement of superficial lymph nodes was common in leprosy, with lymph node biopsies showing a spectrum of changes corresponding to the type of clinical leprosy.5 At the tuberculoid pole lymph nodes are also involved in a significant proportion of patients. An Indian study reported the presence of granulomas in 61.5% and 61.1% of their tuberculoid and borderline tuberculoid cases respectively.6 In the recent years, lymph node involvement in leprosy is less commonly seen except in reactional state.2 In the last decade of the 20th century and first decade of the 21st century, the literature on lymph-node involvement in leprosy is scarce. This could be due to under reporting or a true decrease in the incidence of lymph node leprosy. Since then there have been reports of delay in diagnosis of leprous lymphadenitis and difficulty in differentiating it clinically from lymphoma.79 Probably with the decreasing incidence of leprosy, its complications and ability to be a great masquerader are forgotten. In the case reported here another uncommon presentation of leprosy is discussed. Elephantiasis nostras verrucosa in our patient must have resulted from decreased lymphatic flow due to infiltration of lymph nodes with Mycobacterium leprae laden macrophages. This could have been potentiated by the dependent edema seen in patients with lepromatous leprosy, explained by the autonomic neuropathy affecting small blood vessels of the limbs and accompanying hypoproteinemia.10 Asymptomatic episodes of type II reaction could also have added to its pathogenesis. The appearance of significant lymphedema resulting in elephantiasis reflects a late diagnosis. Thus leprosy should be considered an important cause of secondary lymphedema in tropical countries, to avoid delay in diagnosis.
Conflict of interest
None.
Financial support
None.
Patient consent
Appropriate patient consent was obtained.
Contributor-ship statement
This is to state that Jindal R contributed in planning and conception of this case report. Chauhan P was involved in conception and conduct of this case report. Shirazi N and Chugh R have contributed in patient care and management. Jindal R, Chauhan P, Shirazi N and Chugh R have contributed in drafting of the manuscript and revisions with intellectual inputs. Jindal R, Chauhan P, Shirazi N and Chugh R have approved the final version to be submitted for publication.
References
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