Abstract
Leprosy, caused by Mycobacterium leprae, is a chronic infectious disease that primarily affects the skin, peripheral nerves, and mucous membranes. Despite the availability of multidrug therapy (MDT), leprosy reactions remain a significant cause of morbidity, often leading to severe complications. Type 2 leprosy reaction, or erythema nodosum leprosum (ENL), is characterized by the sudden onset of painful erythematous nodules, fever, and systemic symptoms.1 It typically occurs in patients with lepromatous or borderline lepromatous leprosy, often following the initiation of multidrug therapy (MDT) but when it occurs in treatment naive patients it can be a unique challenge in both diagnosis and management. This case report highlighted 43 years old lady presented with fever, joint pain, skin nodules for 2 months and finally turned out to be a multibacillary type of leprosy with type 2 Lepra reaction.
Introduction
Leprosy, also known as Hansen’s disease, is a chronic infectious disease caused by Mycobacterium leprae and Mycobacterium lepromatosis. Despite being one of the oldest recorded diseases, leprosy continues to pose significant public health challenges, particularly in endemic regions of Asia, Africa, and South America.2 Leprosy is categorized operationally into two main types for treatment purposes: paucibacillary (PB) and multibacillary (MB) forms.3 Early diagnosis and treatment with multidrug therapy (MDT), introduced by the World Health Organization (WHO), have significantly reduced the global burden of leprosy. However, complications such as lepra reactions remain major challenges in clinical management especially in treatment naïve patients.2 Leprosy reactions are typically categorized into two types: Type 1 (reversal reaction) and Type 2 (erythema nodosum leprosum, or ENL). Type 1 reactions are primarily associated with a shift from a Th2 to a Th1 immune response, while Type 2 reactions are linked to the deposition of immune complexes and systemic inflammation. Both types can present with a range of clinical manifestations, including skin lesions, nerve involvement, and systemic symptoms such as fever and malaise.3 This case report aims to highlight a clinical presentation of a leprosy reaction, its diagnostic challenges, and the therapeutic approach.
Through this report, we seek to emphasize the importance of early recognition and management of leprosy reactions to prevent irreversible nerve damage and improve patient outcomes.
Case report
43-year-old lady from Sagaing presented with 1 month history of fever, joint pain and skin lesion. Fever was high grade in nature without chill and rigor. Not associated with respiratory, gastrointestinal and genitourinary symptoms. She felt pain in both big and small joints and noticed skin nodules all over her limbs. No history of fit, limb weakness, oral and genital ulcers. On examination, she was febrile and multiple skin lesion erythema nodosum over both upper and lower limbs. (picture 1) Greater post auricular nerve was palpable and but no hypoesthetic patch was detected. On investigations, neutrophil leucocytosis with ESR-110 and CRP 304 were found. Her liver and renal functions were within normal limit. ANA and ENA profile were negative, hepatic B,C and HIV serostatus were also non-reactive. CXR(PA) and USG (abd) showed no abnormalities. She was clinically suspected with ENL reaction and consulted with specialist from central special skin clinic from YGH. Her split skin smear revealed bacillary index 2+ over left forearm, right cheek and right ear. She was finally diagnosed as erythema nodosum leprosum (ENL) reaction with underlying Hansen’s disease (borderline lepromatous leprosy (BL), multibacillary (MB). She was treated with multidrug therapy for underlying borderline lepromatous leprosy and po prednisolone for lepra reaction. After starting treatment, fever subsided and her skin lesion were significantly resolved. (picture 2) After 3 weeks of hospitalization, she was discharged successfully and planned to attend infection clinic and central special skin clinic regularly.
Fig. 1 Patient’s skin lesion before MDT treatment
Fig. 2 Patient’s skin nodule after MDT treatment
Discussion
Erythema nodosum leprosum (ENL) is a severe Type 2 leprosy reaction that represents a hypersensitivity response to Mycobacterium leprae antigens. It is typically seen in patients with lepromatous or borderline lepromatous leprosy, often occurring after the initiation of multidrug therapy (MDT) for leprosy. 4 ENL is characterized by the sudden onset of tender, erythematous nodules on the skin, accompanied by systemic symptoms such as fever, malaise, and in some cases, significant nerve involvement. Although ENL is a well-known complication of leprosy, its management remains complex, particularly in treatment-naive patients, as observed in the present case. In this report, a 43-year-old female with newly diagnosed lepromatous leprosy developed an acute ENL reaction, which had not been previously treated. The onset of ENL reactions can vary, but they are typically precipitated by a shift in immune response, often occurring during or shortly after the commencement of MDT.5 The patient in this case presented with classic clinical features of ENL, including painful erythematous nodules and systemic symptoms, which are highly indicative of this reaction. The diagnosis was confirmed based on clinical findings and the exclusion of other possible causes of erythematous skin lesions and systemic inflammation.Management of ENL in treatment-naive patients poses a unique challenge, as early recognition and intervention are critical to prevent long-term complications. The cornerstone of treatment for ENL includes systemic corticosteroids and multidrug therapy which are used to control inflammation and prevent nerve damage.7 In this case, the patient responded well to treatment with a significant reduction in systemic symptoms and improvement in skin lesions within a short period. The management of ENL in treatment-naive patients requires careful consideration of the severity of the reaction, the patient’s underlying leprosy status, and the potential side effects of treatment. While corticosteroids are effective in reducing inflammation and preventing nerve damage, their long-term use is associated with significant side effects such as hyperglycemia, osteoporosis, and infection. Therefore, these medications must be used judiciously, and close monitoring is essential throughout the course of treatment. For treatment-naive patients, initiating appropriate therapy as soon as ENL is diagnosed can significantly improve outcomes and reduce the risk of permanent complications. Regular follow-up visits are essential to monitor for recurrence of ENL, as these reactions can recur even after successful initial treatment.
Conclusion
ENL is a potentially debilitating complication of leprosy that requires prompt recognition and appropriate management, particularly in treatment-naive patients. Early intervention with corticosteroids and/or thalidomide can effectively control symptoms, prevent long-term nerve damage, and improve patient quality of life.7 Furthermore, this case highlights the importance of early detection and treatment of ENL, as delayed management can lead to irreversible nerve damage, disability, and deformities.
Reference
1. Walker, S. L., Lockwood, D. N. J. (2008). “The clinical and immunological features of leprosy.” British Medical Bulletin, 77-78(1), 103–121.
2. World Health Organization (2018). Guidelines for the diagnosis, treatment, and prevention of leprosy.
3. Job, C. K., Nandakumar, G. (2001). “Erythema Nodosum Leprosum: A Clinicopathological Study.” International Journal of Leprosy and Other Mycobacterial Diseases, 69(4), 325–335.
4. Lockwood, D. N. J. (2000). “Leprosy and Erythema Nodosum Leprosum (ENL).” Current Opinion in Infectious Diseases, 13(2), 141–145.
5. Scollard, D. M., Adams, L. B., Gillis, T. P., et al. (2006). “The continuing challenges of leprosy.” Clinical Microbiology Reviews, 19(2), 338–381.
6. Davison’s Principle and practice of Medicine, 24th edition.
7. Rea, T. H., Levan, N. E. (1975). “Erythema Nodosum Leprosum in a General Hospital.” Archives of Dermatology, 111(12), 1575–1580
Author Information
Aye Mya Theingi Win1, Khin Rupar Ko2, Aye Aye Win3, May Zabe4, Nyunt Thein5
1. Consultant physician, Tropical and Infectious Diseases Department, Yangon General Hospital
2. Professor, Tropical and Infectious Diseases Department, Yangon General Hospital
3. Associated Professor, Tropical and Infectious Diseases Department, University of Medicine (1), Yangon
4. Consultant physician, Tropical and Infectious Diseases Department, Yangon General Hospital
5. Senior Consultant physician, Former Head of Department of Medicine, Emeritus Professor of Medicine, University of Medicine (1), Yangon
