Abstract
Erythema nodosum leprosum (ENL) is a severe multisystem immune mediated complication of borderline lepromatous leprosy and lepromatous leprosy. ENL is associated with skin lesions, neuritis, arthritis, dactylitis, eye inflammation, osteitis, orchitis, lymphadenitis and nephritis. The treatment of ENL requires immunosuppression, which is often required for prolonged periods of time and may lead to serious adverse effects. ENL and its treatment is associated with increased mortality and economic hardship. Improved, evidence-based treatments for ENL are needed; however, defining the severity of ENL and outcome measures for treatment studies is difficult because of the multiple organ systems involved. A cross-sectional study was performed, by the members of the Erythema Nodosum Leprosum International STudy (ENLIST) Group, of patients with ENL attending seven leprosy referral centres in Brazil, Ethiopia, India, Nepal, the Philippines and the United Kingdom. We systematically documented the clinical features and type of ENL, its severity and the drugs used to treat it. Patients with chronic ENL were more likely to be assessed as having severe ENL. Pain, the most frequent symptom, assessed using a semi-quantitative scale was significantly worse in individuals with “severe” ENL. Our findings will determine the items to be included in a severity scale of ENL which we are developing and validating. The study also provides data on the clinical features of ENL, which can be incorporated into a definition of ENL and used for outcome measures in treatment studies.
Highlights
Leprosy reactions are a significant cause of morbidity
Leprosy is caused by a bacterium and is curable with a combination of antibiotics known as multi-drug therapy, which patients take for six or 12 months
A significant proportion of leprosy patients experience severe inflammation in their skin, nerves and other organs known as erythema nodosum leprosum (ENL)
Summary
Erythema nodosum leprosum (ENL) is an immunological complication affecting individuals with lepromatous leprosy (LL)[1]. ENL affects approximately 50% of individuals with LL and 5–10% of borderline lepromatous (BL) leprosy patients [1, 2]. ENL is a multisystem disorder characterised by the occurrence of crops of tender skin lesions. The histology of ENL lesions classically shows an intense perivascular infiltrate of neutrophils throughout the dermis and subcutis [3]. The pathophysiology of ENL has not been fully elucidated though immune complexes and cellular immunity mechanisms have been reported be involved. ENL has some features of an immune complex mediated disease. Direct immunofluorescence studies have demonstrated granular deposits of immunoglobulin and complement in the dermis in ENL lesions but not in those of uncomplicated LL disease [4]. There is evidence of T lymphocyte and macrophage activation and expression of mRNA for TNFα and IL12 in the skin [5]
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