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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 7  |  Issue : 3  |  Page : 84-86

An enigmatic case of pure neuritic leprosy with trophic ulcers


Department of General Medicine, M.R Medical College, Kalaburagi, Karnataka, India

Date of Submission19-Sep-2018
Date of Decision22-Nov-2018
Date of Acceptance20-Dec-2018
Date of Web Publication15-Jul-2019

Correspondence Address:
Dr. Mohd Zeeshan Ali
Department of Internal Medicine, M.R. Medical College, Kalaburagi, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AJIM.AJIM_20_19

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  Abstract 

Leprosy is one of the most common diseases of peripheral nerves. In pure neuritic leprosy, skin along the distribution of the affected nerve is usually hypo-anesthetic or anesthetic, and as a rule, no classical leprosy skin lesions/patches should be present. However, depending on the severity of sensory and autonomic dysfunction, there could be a variable degree of hypo/anhidrosis, xerosis, fissuring, and ulcers along its distribution. Diagnosis of leprosy in the absence of typical dermatological features is difficult and requires histopathological confirmation using nerve biopsy. We report a case of leprosy with peripheral neuropathy without skin lesions. Nerve biopsy showed chronic inflammatory cell infiltration, epithelioid-cell granulomas, and the presence of Mycobacterium leprae on Modified Ziehl-Neelson stain. Hence, in leprosy prevalent countries like India, this form of leprosy should be thoroughly investigated, especially in patient without skin changes and presenting with trophic ulcers. It was a diagnostic challenge requiring high degree of clinical suspicion and thorough investigations.

Keywords: Leprosy, pure neuritic, trophic ulcers


How to cite this article:
Ali MZ, Biradar S, Sumangala S. An enigmatic case of pure neuritic leprosy with trophic ulcers. APIK J Int Med 2019;7:84-6

How to cite this URL:
Ali MZ, Biradar S, Sumangala S. An enigmatic case of pure neuritic leprosy with trophic ulcers. APIK J Int Med [serial online] 2019 [cited 2019 Aug 19];7:84-6. Available from: http://www.ajim.in/text.asp?2019/7/3/84/262746




  Introduction Top


Leprosy is one of the common treatable dermato-neurologic disease affecting the skin and peripheral nerves. Although the prevalence of disease is decreasing, leprosy represents one of the major public health problems mainly in India and Brazil.[1],[2],[3] The diagnosis of leprosy is straight forward when both cutaneous and neurological involvements are present together. Patient with leprosy may have only nerve involvement without obvious primary skin lesions and is called pure neuritic leprosy (PNL).[4],[5],[6] Neuritis in leprosy is usually a subacute, demyelinating, and nonremitting event involving cutaneous nerves and larger peripheral nerve trunks. Although “polyneuritic” type of PNL is reported, some workers prefer to call it “moneuritis multiplex summation.”[7] Patients with “pure neuritic” form of leprosy are frequently misdiagnosed because of the absence of skin lesions.[8] We report a case of unusual presentation of neuritic leprosy with trophic ulcers.


  Case Report Top


A 30-year-old female patient presented with a history of numbness and tingling sensations of both upper limbs up to the elbow for 6 months and ulcers on fingers of both hands for 2 months. There was no history of weakness of upper limbs. No history suggestive of autonomic disturbances was present. The patient was nondiabetic and nonalcoholic, and there was no history of high-risk sexual behavior. She consumed a mixed diet.

There was no history of skin lesions or contact with any person suffering from leprosy. On general physical examination, the patient was afebrile. Vitals were stable. Trophic ulcers were present on fingers of both upper limbs on both dorsal and palmar surfaces, as shown in [Figure 1], [Figure 2], [Figure 3]. Examination of skin did not reveal any skin lesions suggestive of leprosy. The ulnar nerve was thickened in both the upper limbs.
Figure 1: Trophic ulcers on palmar surface of the hands involving the digits and palm both

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Figure 2: Ulcers on the dorsal aspect of the hands

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Figure 3: Ulcers on the palmar aspect of the hands

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On central nervous system examination, the patient was conscious and well oriented. Speech was normal. Motor system examination showed the presence of weakness of adduction and abduction movements of the fingers (mild weakness of palmar and dorsal interossei) and weakness of opposition of thumb (opponens pollicis) of the right hand and weakness of abduction (dorsal interossei) in the left hand. There was no wasting of small muscles of hands. On examination for sensory function, there was distal glove and stocking distribution on both upper limbs with thermal and pinprick anesthesia and preservation of proprioception and vibration. Reflexes were diminished in upper limbs and were normal in both lower limbs. Both plantar reflexes were flexor. Other systemic examination did not reveal any abnormality.

Laboratory investigations

Complete blood picture was normal. Erythrocyte sedimentation rate was 88 mm at the end of the 1st h. Liver functions and renal functions tests were normal. Random blood sugar was 81 mg/dl. The venereal disease research laboratory was negative. Doppler study of both upper limbs was normal. Chest X-ray was normal. Clinical suspicion was mixed sensory-motor peripheral neuropathy, vasculitic, and autoimmune origin.

Skin biopsy was taken from the right forearm. Histopathological examination revealed intense lymphocytic infiltration predominantly in endoneurium and perineurium along with the presence of granulomas and plasma cells. Perivascular lymphocytic infiltration was absent. Special stain-Modified Ziehl–Neelsen stain showed few acid-fast bacilli (AFB) (+), Toluidine blue – showed focal areas of demyelination. The Modified Ziehl–Neelsen stain of smears from finger ulcers was negative for AFB. The diagnosis of PNL was made.

Nerve conduction study was performed on nerves of the upper and lower limbs, which revealed delayed distal latencies in the left ulnar nerve. Compound muscle action potentials were low in amplitude in the bilateral median and ulnar nerves and absent in bilateral tibial nerves. Conduction velocities were normal. Sensory nerve action potentials were obtained from the bilateral median, ulnar, superficial peroneal nerve, and sural nerves. The final impression of the study was asymmetric sensorimotor polyneuropathy.

The patient was started on tab clofazimine and dapsone. The patient was improving on follow up, and the neuropathy has improved, and the ulcers are healed after 5 months of initiation of treatment.


  Discussion Top


PNL has always been an enigma due to its ambiguities. The widely accepted classification for leprosy proposed by Ridley–Jopling based on clinical, histological, and immunological criteria does not include “neuritic leprosy” in its five-group system. Previously, it was called as polyneuritic type of leprosy. The Indian Association of Leprologist's classification recognizes “pure neuritic leprosy” as a distinct subgroup. Albert Neisser, in 1903 for the first time mentioned a “neural type of leprosy” and added, “lepra nervorum” to the “nodular” and “anesthetic” forms of leprosy already accepted until then.[9] It is important to maintain PNL as a subgroup as it constitutes a good percentage of leprosy cases reported from India, which contributes to more than half of global leprosy numbers. A high proportion of these patients present with Grade 2 disability at the time of initial reporting itself due to the early nerve involvement.[2] The ulcers in leprosy occur by the direct action of Mycobacterium leprae on the peripheral nerves, with changes in the sensory, autonomic and motor fibers (neuropathic ulcers). Less commonly, it is due to direct invasion of bacilli in the vascular endothelium, causing vasculitis, cutaneous necrosis, and ulcers.[10]

Peripheral nerve enlargement is not consistently present. In this patient, pure polyneuritic form of leprosy was histopathologically diagnosed. Although skin lesions are absent by definition, when skin biopsies were performed from the skin along the distribution of the affected nerve, a proportion of patients demonstrates leprosy pathology, revealing subclinical skin involvement.

In addition, on follow-up, skin lesions are noted to develop in up to 20% of PNL cases, indicating its progression to manifest cutaneous disease.[11] Over the decades, the confirmation of diagnosis of PNL has been subjective, however, with the arrival and use of high-resolution ultrasonography for nerve imaging, we have a tool not only to objectively measure and record the nerve thickening but also to assess the morphological alterations in the nerve including echotexture, fascicular pattern, and vascularity.

Histopathological examination of a peripheral nerve biopsy is the gold standard for the diagnosis of PNL, though most of the cases are diagnosed on clinical findings and electroneuromyographical findings. Clinically, peripheral nerves may be thickened in PNL. Nerve conduction studies may show sensory or sensorimotor, axonal, demyelinating, or mixed axonal and demyelinating multiple mononeuropathy or symmetrical confluent peripheral neuropathy.

The management of PNL requires multidrug therapy along with an appropriate dose of systemic corticosteroids, for both acute and silent neuritis. Measures for pain relief, self-care of limbs, and physiotherapy are important to prevent as well as manage disabilities in this group of patients.


  Conclusion Top


We report this case because this form of leprosy is still active in India, and its diagnosis is usually missed. Peripheral nerve biopsy is one of the best procedures for patients with small fiber neuropathy without skin lesions. There is a need to pay more attention to this form of leprosy to diagnose and treat patients earlier and to prevent sequelae. Unusual presentation of leprosy should be clinically suspected as PNL. It should be considered as one of the differential diagnoses of peripheral neuropathy and thoroughly investigated with nerve biopsy followed by histopathological examination. Even during postelimination era, new cases of neuritic leprosy continue to be reported indicating the presence of infection in the community.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Noordeen SK. Elimination of leprosy as a public health problem: Progress and prospects. Bull World Health Organ 1995;73:1-6.  Back to cited text no. 1
    
2.
Talwar S, Jha PK, Tiwari VD. Neuritic leprosy: Epidemiology and therapeutic responsiveness. Lepr Rev 1992;63:263-8.  Back to cited text no. 2
    
3.
Andrade V, Militao de Albuquerque MD, Chagastelles Sabroza P. The importance of operational factors for the interpretation of indicators in the Hansen's disease endemic in Brazil. Acta Leprol 1997;10:131-9.  Back to cited text no. 3
    
4.
Girdhar BK. Neuritic leprosy. Indian J Lepr 1996;68:35-42.  Back to cited text no. 4
    
5.
Jennekens FG, van Brakel WH. Neuropathy in leprosy. In: Latov N, Wokke JH, Kelly JJ Jr., editors. Immunological and Infectious Disease of Peripheral Nerves. Cambridge: Cambridge University Press; 1998. p. 319-39.  Back to cited text no. 5
    
6.
Mahajan PM, Jogaikar DG, Mehta JM. A study of pure neuritic leprosy: Clinical experience. Indian J Lepr 1996;68:137-41.  Back to cited text no. 6
    
7.
Nascimento OJ. Leprosy neuropathy: Clinical presentations. Arq Neuropsiquiatr 2013;71:661-6.  Back to cited text no. 7
    
8.
Rao PN, Suneetha S. Pure neuritic leprosy: Current status and relevance. Indian J Dermatol Venereol Leprol 2016;82:252-61.  Back to cited text no. 8
[PUBMED]  [Full text]  
9.
Scollard DM. The biology of nerve injury in leprosy. Lepr Rev 2008;79:242-53.  Back to cited text no. 9
    
10.
Andrade PJ, Oliveira PC, Messias SS, Nery JA. Indolent ulcers: Leprosy as differential diagnosis. Hansen Int 2013;38:154.  Back to cited text no. 10
    
11.
Suneetha S, Arunthathi S, Chandi S, Kurian N, Chacko CJ. Histological studies in primary neuritic leprosy: Changes in the apparently normal skin. Lepr Rev 1998;69:351-7.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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