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Leprosy is one of the most common causes of nontraumatic peripheral neuropathy in the developing world. The causative agent, Mycobacterium leprae, has a predilection for Schwann cells, where the organism multiplies unimpeded by organism-specific host immunity, resulting in destruction of myelin, secondary inflammatory changes, and destruction of the nerve architecture. The cardinal diagnostic features of leprosy are anesthetic skin lesions, neuropathy, and positive skin smears for the bacilli. However, patients may rarely present without skin lesions in pure neuritic leprosy. Electrodiagnostic findings early in the disease reveal demyelinating features, such as slowing of conduction velocity and prolongation of latencies, but as the disease progresses secondary axonal damage commonly ensues. Electrodiagnostic studies are also useful to monitor for toxicity secondary to therapy, particularly thalidomide-associated neuropathy. Nerve biopsy of a sensory cutaneous nerve is sometimes essential to confirm a diagnosis of leprosy. Significant advances in understanding of the pathogenesis, mapping of the genome, and other advances in molecular biology may result in better preventive and therapeutic modalities, and the goal of eradicating leprosy as a global problem may yet be realized.

The World Health Organization established a goal in 1991 of "elimination of leprosy as a public health problem by the year 2000." Although prevalence rates of leprosy have decreased in many geographic areas, it is clear that in some countries where leprosy is endemic, such as Brazil and India, this goal will not be reached. Leprosy is rare in the United States, but 85% of detected cases are in immigrants in whom the disease may mimic many common dermatologic and neurological entities, leading to delay of diagnosis. The statuses of polymerase chain reaction analysis, serological testing, and vaccines are reviewed. Effective multidrug therapy and prevention of permanent damage to nerves by early recognition and treatment will help prevent residual disabilities. This update reviews what is known about the pathophysiology and treatment of leprosy. Increased awareness will lead to earlier recognition, diagnosis, and treatment.