Pure Neuritic Hansens: A Rare Case Report

 

Article By:

Merline Augustine

Priyanka Mashelkar

R. G. Wiseman Pinto

  

Abstract

Pure Neuritic Hansens is a rare presentation of spectrum of Hansens . A case report is presented with differential diagnosis to be considered.

 

Introduction :

Hansens Disease is a chronic inflammatory disease caused by an acid fast bacilli “Mycobacterium Leprae”. The bacilli mostly affects the peripheral nerves and has an affinity to Schwann cells and axons. Pure Neuritic Hansens implies there are no other specific skin lesions of cutaneous Hansens such as hypopigmented patches, plaques, papules elsewhere.[1]

 

Case report :

A 30-year-old male presented to the Out Patient Department of Goa Medical College with a chief complaint of reduced sensation of left upper limb for a duration of one year. There were no other complaints.

Patient was non alcoholic, non diabetic and there was no history of intake of any chronic medications.

On general examination all the vitals were stable. There was drying of skin of left upper limb. There were no other skin lesions suggestive of Hansens.

On neurological examination, patient was conscious, alert, oriented to time, place and person. The sensory functions such as touch, temperature were impaired on the left upper limb. Reflexes were within normal limits.

Systemic examination did not reveal any abnormality.

Investigations: HIV, VDRL were nonreactive. Blood investigations did not reveal any abnormality.

 

Patient underwent a nerve biopsy. Left ulnar cutaneous nerve was biopsied and sent to Department of Pathology in Goa Medical College. Nerve was subjected to histopathological examination. Microscopic examination following routine hematoxylin and eosin staining revealed dense lymphocytic infiltration around perineurium. Special stain using modified Fite Faraco revealed positivity for acid fast bacilli. Thus diagnosis of Pure Neuritic Hansens was made.

 

Discussion:

Pure Neuritic Hansens is a rare presentation of Hansens disease seen in about 4-18% of Hansens cases in India.[2]

 

Clinical features :

Mostly the patients present with reduced or loss of sensation in the area supplied by affected nerve. The nerve involved may be thickened or beaded. The skin changes include dryness (xerosis), ulcers along the distribution of nerve. As per definition of Pure Neuritic Hansens, there should be no other classical skin lesions of Hansens.

 

Investigations :

Fine Needle Aspiration, Polymerase Chain Reaction, Nerve Conduction Studies, MRI and High Resonance Ultrasonography can be done. However nerve biopsy remains gold standard. Nerves usually biopsied are superficial radial nerve, ulnar cutaneous nerve and sural nerve.

Microscopy: Non specific perineural inflammation consisting of lymphocytes, plasma cells. There may be epithelioid cell granulomas as well. Perineural fibrosis, pus formation and calcium deposition may be also seen. Modified Fite Faraco staining demonstrates acid fast bacilli. (Figure 1 and 2)

 

TC- Jan 2017 - 013 - Figure 1. Cross section of a nerve TC- Jan 2017 - 014 - Figure 2. Cross section of a nerve

 

Differential diagnosis includes the following[3]:

  • Autoimmune
  • Diabetic neuropathy
  • Amyloidosis
  • HIV
  • Sarcoidosis
  • Neurofibromatosis
  • Hereditary sensory neuropathy

 

TC- Jan 2017 - 015 - Writers Art pg 27

References:

  1. Menicucci L, Miranda A, Antunes S, Jardim M, Nery J, Sales A, et al. Microscopic leprosy skin lesions in primary neuritic leprosy. J Am Acad Dermatol 2005; 52:648-652.
  2. Sharma VK, Malhotra AK. Leprosy: Classification and clinical aspects. In: Valia RG, Valia AR, editors. IADVL Text Book of Dermatology. 3rd ed. Mumbai: Bhalani Publishing House; 2008. p. 2032-69.
  3. Ramanujam K.Differential Diagnosis of Pure Neuritic Leprosy. Indian J Lepr. 2005 Jul-Sep;77(3):277-9.