Abstract

Leprosy is a chronic infection caused by Mycobacterium leprae that affects the peripheral nerves, skin, and potentially other organs [1]–[5]. Although the worldwide prevalence of leprosy has decreased in the era of multi-drug therapy (MDT), the global detection of new cases of leprosy remains a concern, with more than 250,000 new cases of leprosy reported in 2007 [3]. The precise mechanism of transmission of leprosy has not been conclusively defined; however, it is likely that this occurs through respiratory secretions by untreated borderline lepromatous and polar lepromatous cases [1],[5]. Target cells of infection are macrophages, histiocytes in the skin, and the nonmyelinating and myelinating Schwann cells in the peripheral nerve, leading to axonal dysfunction and demyelination [6]. Nerve injury plays a central role in the pathogenesis of leprosy, leading to functional impairment and deformity of hands and feet and the eyes [1],[6]. Leprosy is diagnosed by definite loss of sensation in a hypopigmented or reddish skin patch, a thickened peripheral nerve with loss of sensation and muscle weakness in the affected nerve, and presence of acid-fast bacilli on skin smear or biopsy [1],[4]. The immunological response to M. leprae mounted by the host will determine the different potential clinical states. The Ridley-Joplin system uses clinical and histopathological features and the bacteriologic index and includes the polar categories (lepromatous [LL] and tuberculoid [TT]) and the borderline states (borderline tuberculoid [BT], borderline borderline [BB], and borderline lepromatous [BL]) [1] (Table 1). In the polar tuberculoid category, a Th1 type cell–mediated immune response with a low bacterial load is seen. Lepromatous states are characterized by low cell-mediated immunity and a higher bacterial load [5] (Table 1). Clinically, patients with tuberculoid leprosy have a single or very few hypopigmented macules or plaques with a raised edge; they are dry, scaly, hairless, and have reduced sensation; and only a few peripheral nerves are commonly enlarged [1]. Lepromatous leprosy is characterized by widely and symmetrically distributed skin macules, nodules, erythematous papules, and diffuse skin infiltration; thickened peripheral nerves are more frequently identified. Borderline states represent a mixture of signs and symptoms of the polar categories [1]. Table 1 World Health Organization System and Ridley-Joplin Classification and Type of Reaction.

Highlights

  • A 31-year-old Brazilian male living in the United States for the previous four years presented with progressive crops of new nontender nodules on all four extremities over a 16-month period (Figures 1 and 2)

  • The patient was diagnosed with lepromatous leprosy, using the Ridley-Joplin staging system [1], or multibacillary leprosy per the World Health Organization (WHO) staging [2,3,4,5] (Table 1)

  • We recommended that he continue his multi-drug therapy (MDT) until his bacterial index (BI) decreased below 2

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Summary

Description of Case A

A 31-year-old Brazilian male living in the United States for the previous four years presented with progressive crops of new nontender nodules on all four extremities over a 16-month period (Figures 1 and 2). He had referred numbness in the lower extremities He had thickened bilateral ulnar nerves with mild sensory loss by monofilament testing in the ulnar nerve and peroneal nerve territories without any nerve tenderness identified. The patient was diagnosed with lepromatous leprosy, using the Ridley-Joplin staging system [1], or multibacillary leprosy per the World Health Organization (WHO) staging [2,3,4,5] (Table 1) He was started on MDT consisting of dapsone 100 mg PO daily, rifampin 600 mg PO daily, and clofazimine 50 mg PO daily. Based on the clinical history and signs, our patient was diagnosed with a type 2 leprosy reaction [5] (Table 2) He was treated with 60 mg of oral prednisone daily tapered over a 12-week period [4]. A repeat skin biopsy performed 12 months after commencing MDT showed decreasing numbers of acid-fast bacilli, and skin smears showed a decreasing BI

What Are Leprosy Reactions and How Are They Diagnosed?
Occurs in BL and LLa
Unusual Clinicalc Steroidse
Clinical Manifestations
Description of Case B
Key Learning Points
Findings
Discussion of Cases A and B
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