Digital Gangrene in Hansen’s DiseaseAn Atypical Presentation

Dr. Aditi Singh

MBBS, DDVL, FAGE

Consultant Dermatologist and Aesthetician

Dr Aditi Singh's Skin Clinic, Raipur Government District Hospital, Raipur

Abstract

Digital gangrene in upper limbs may be due to systemic sclerosis, trauma, connective tissue disorders or vasculitic disorders.[1] Leprosy as an infectious cause of gangrene, is rare and gangrene associated with leprosy is unusual except in the Lucio variant. Our patient presented with no features of connective tissue disease or vasculitis. She also tested negative for the serology done to rule out the same. Also, she did not show any classical clinical features of Lucio leprosy. Further, she was also pregnant at the time of presentation, confirmed by a positive Urine Pregnancy Test (UPT). We are reporting this case as it was a very atypical presentation of leprosy coupled with an uncommon subset of patient population: pregnancy. She is currently under follow up, and on Multi Drug Therapy for leprosy.

Keywords: leprosy, gangrene, pregnancy

Introduction

Leprosy is a chronic infectious disease caused by a type of bacteria, Mycobacterium leprae.

The disease predominantly affects the skin and peripheral nerves. As per WHO, elimination of leprosy as a public health problem globally (defined as prevalence of less than 1 per 10000 population) was achieved in 2000. But it still remains an important public health problem in many parts of the world.[2] Leprosy presenting as digital gangrene is unusual [3] and this case is being reported due to its peculiar presentation and that too in a pregnant lady.

Case report

A 22 year old female patient presented to the Dermatology OPD of District Hospital, Raipur with the chief complaint of blackish discoloration of right index finger since 3 months. It was insidious in onset, associated with pain. She gave a history of an ulcer on the tip of the finger which was apparently surgically debrided at a peripheral hospital, but there was no improvement, after which she was referred to our centre. There was no history of similar complaints in any other parts of the body. She also gave history of an abortion at 7 months of pregnancy, prior to the onset of blackish discoloration of the finger. She denied any complaints during pregnancy. There was no history of smoking, facial rash, photosensitivity, oral ulcers, joint pains or fever prior to onset of present symptoms. There was a history of 2 months of amenorrhoea along with the presenting complaints, which was subsequently confirmed to be another pregnancy by UPT.

She also complained of hypoesthesia in her right thumb and index finger and adjoining areas of palm since 3 months. There was no history of any other such patches on the body. There was no history of painful skin lesions in the past. There was no history of loss of hair from any part of the body. There was no family history of Hansen’s disease.

On examination, right finger showed blackish discoloration in the distal half, with a tapering end (possibly due to autoamputation) and an illdefined fibrotic hypopigmented patch on the dorsum of right thumb. Sensations were diminished in the right Median Nerve distribution on the palmar aspect of the right hand and right Ulnar Nerve thickened and non-tender. There were no other patches present elsewhere on the body.

Given the history and clinical presentation, we zeroed in on Hansen’s disease with peripheral vascular disease as a working diagnosis and got her evaluated accordingly.

Her Total Leucocyte Count (TLC), Differential Leucocyte Counts (DLC), Haemoglobin, Erythrocyte Sedimentation Rate

(ESR), Liver Function test (LFT), Renal Function Test (RFT), Urine Routine and Microscopy, Thyroid Function Test were all within normal limits. Anti-Nuclear Antibody (ANA), anti-double stranded DNA (anti-dsDNA), anti Phospholipid Antibodies (APLA), Anticardiolipin antibodies (ACLA) were all negative. Arterial Doppler of right upper limb was normal. Thus vascular and auto-immune causes of gangrene were ruled out. As mentioned earlier, UPT was positive. Slit-skin smears from lobes of both ears were 1+. A diagnosis of Hansen’s disease with pregnancy was made and she was started on Pauci-Bacillary Multi-Drug Therapy (PB-MDT). A B

Discussion

When the woman first presented to us in the Dermatology OPD, she was essentially a case of digital gangrene under evaluation. A concomitant history of 2 months amenorrhoea prompted us to test for pregnancy which came positive, and a history of abortion in recent past made us think of Anti Phospholipid Antibody Syndrome, which we ruled out by negative serology. At this point, digging deeper into the history we found that she had areas of hypoesthesia in the ipsilateral hand. Hence a differential diagnosis of peripheral vascular disease with Hansen’s disease was considered and work-up done accordingly. After getting the laboratory results, we ruled out vaso-occlusive disease and suspected Hansen’s disease. But Hansen’s disease presenting with gangrene is unusual.[3] This presentation prompted us to consider Lucio leprosy since it is the form of leprosy that presents with gangrene. However Lucio Leprosy is a variant of Lepromatous leprosy and is considered a necrotizing panvasculitis.[2] Clinically, our patient did not seem to have Lepromatous leprosy and in light of the fact that she refused for a skin biopsy, we could not confirm. Also, it did not seem like a classical presentation of Luciovariant of leprosy. Gangrene in leprosy (other than Lucio leprosy) is postulated to have various mechanisms.[1]

Vessel wall changes such as intimal thickening and medial infiltration, embolization of Virchow cells or nerve trunk hypertrophy due to the lepromatous process can cause arterial occlusion in osteoligamentous channels. Other possible mechanisms may be vasospasm due to irritation of sympathetic fibres and lastly, the Lucio phenomenon.[4]

This case report highlights that gangrene as a clinical entity must be evaluated thoroughly and prompts us to be alert about the infectious cause of gangrene (leprosy) especially in the Indian scenario,where it is still a public health issue at large.

References

1. Singh S, Chand G, Charan S, Arora S, Singh P. Peripheral arterial disease and digital gangrene: a rare presentation of diabetic hand syndrome. J Clin Diagn Res. 2013Oct;7(10):2286-7.

2. Nunzie E, Ortega Cabrera LV, Macanchi Moncayo FM, Ortega Espinosa PF, Clapasson A, Massone C. Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies. Lepr Rev. 2014 Sep;85(3):194-200.

3. Akerkar SM, Bichile LS. Leprosy & gangrene:a rare association; role of antiphospholipid antibodies. BMC Infect Dis. 2005 Sep21;5:74.

4. El-Anany G, Nada H, Nagui N, Shalaby S, Sany E, Elmongy N, Nada A.Finger gangrene: an unusual presentation of leprosy .J Egypt Womens Dermatol Soc 2019;16:68-70.

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