Hansen’s disease (HD) is the most common cause of neuropathy in the developing and underdeveloped countries.
In 1903, Albert Neisser described a "neural type of leprosy/lepra nervorum" for the first time and added the same to the already accepted “nodular” and “anesthetic” forms of leprosy.
The Indian Association of Leprologists (IALs) included the distinct form of "neural leprosy" in their official six group classification in 1955 and named it "polyneuritic leprosy."
Hansen’s neuritis (HN) clinically manifests as thickened or tender nerves leading to sensory, motor, or autonomic disturbances and formation of trophic ulcers as well as loss of tissue.
There are no WHO guidelines for subclassification of pure/primary neuritic HD (PNHD).
Mononeuritis multiplex is the most frequent clinical manifestation of PNHD.
Nerve biopsy is essential for the diagnosis of PNHD, which exhibits no skin lesions.
Biopsy: In lepromatous HN, there is an uneven involvement of fascicles with relative preservation of the overall architecture. Macrophages and Schwann cells filled with organisms and debris (foamy cells) appear in the epineurium, endoneurium, and perineurium, accompanied by a strikingly mild local reaction. Perivascular cuffing is common but never reaches the level of true vasculitis, except in acute reactions. Bacilli abound and are found within macrophages (“lepra cells”) as intracellular “globi” containing dozens or even hundreds of bacteria. In tuberculoid HN, the hallmark of peripheral nerve involvement is an exuberant granulomatous reaction that severely damages the fascicular architecture. Granulomata are accompanied by multinucleated giant cells, and variable amounts of lymphocytes and plasma cells. Organisms are not detectable in polar tuberculoid HN.
References
Bilbao JM, Schmidt RE. Biopsy Diagnosis of Peripheral Neuropathy. Springer International Publishing, 2015.
Jaiswal N, Chakraborti S, Nayak S et al. Hansen's Neuritis Revisited – A Clinicopathological Study. J Neurosci Rural Pract. 2018; 942–55.
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