Erythema multiforme in leprosy

Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

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“…Target lesions are typical. 6 , 7 In non-typical cases, the condition may be confused with Sweet's syndrome-like reaction.…”

Section: Discussionmentioning

confidence: 99%

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Chiaratti

Daxbacher

Neumann

et al. 2016

An. Bras. Dermatol.

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“…T2R patients were clinically diagnosed as ENL (n = 19) or erythema multiforme (EM; n = 4) according to the type of skin lesions present at the dermatological examination. The so called “iris” or target lesion was considered a primary characteristic of EM, while dermal erythematous nodules a characteristic of ENL [3,16]. Reaction occurred before (n = 3), during (n = 9), or after ending MDT (n = 11).…”

Section: Methodsmentioning

confidence: 99%

Neutrophil extracellular traps contribute to the pathogenesis of leprosy type 2 reactions

et al. 2019

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Up to 50% of patients with the multibacillary form of leprosy are expected to develop acute systemic inflammatory episodes known as type 2 reactions (T2R), thus aggravating their clinical status. Thalidomide rapidly improves T2R symptoms. But, due to its restricted use worldwide, novel alternative therapies are urgently needed. The T2R triggering mechanisms and immune-inflammatory pathways involved in its pathology remain ill defined. In a recent report, we defined the recognition of nucleic acids by TLR9 as a major innate immunity pathway that is activated during T2R. DNA recognition has been described as a major inflammatory pathway in several autoimmune diseases, and neutrophil DNA extracellular traps (NETs) have been shown to be a prime source of endogenous DNA. Considering that neutrophil abundance is a marked characteristic of T2R lesions, the objective of this study was to investigate NETs production in T2R patients based on the hypothesis that the excessive NETs formation would play a major role in T2R pathogenesis. Abundant NETs were found in T2R skin lesions, and increased spontaneous NETs formation was observed in T2R peripheral neutrophils. Both the M. leprae whole-cell sonicate and the CpG-Hlp complex, mimicking a mycobacterial TLR9 ligand, were able to induce NETs production in vitro. Moreover, TLR9 expression was shown to be higher in T2R neutrophils, suggesting that DNA recognition via TLR9 may be one of the pathways triggering this process during T2R. Finally, treatment of T2R patients with thalidomide for 7 consecutive days resulted in a decrease in all of the evaluated in vivo and ex vivo NETosis parameters. Altogether, our findings shed light on the pathogenesis of T2R, which, it is hoped, will contribute to the emergence of novel alternative therapies and the identification of prognostic reactional markers in the near future.

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“…It seems that with the evolution of the ENL lesions, the number of lymphocytes and plasmocytes increases, while the number of neutrophils and eosinophils decreases (11, 15–18). Vascular abnormalities (endothelial swelling, edema, and angiogenesis) are consistently observed in acute stage of ENL lesions and reduced after anti-reactional treatment (11, 18–20). The ulcerated form, called necrotizing ENL, demonstrates similar, though more intense, histological findings and leukocytoclastic vasculitis is observed (17, 21, 22) (Figure 2).…”

Section: Introductionmentioning

confidence: 99%

Neutrophils in Leprosy

et al. 2019

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Leprosy is an infectious disease caused by the intracellular bacillus Mycobacterium leprae that mainly affects the skin and peripheral nerves. One of the most intriguing aspects of leprosy is the diversity of its clinical forms. Paucibacillary patients are characterized as having less than five skin lesions and rare bacilli while the lesions in multibacillary patients are disseminated with voluminous bacilli. The chronic course of leprosy is often interrupted by acute episodes of an inflammatory immunological response classified as either reversal reaction or erythema nodosum leprosum (ENL). Although ENL is considered a neutrophilic immune-complex mediated condition, little is known about the direct role of neutrophils in ENL and leprosy disease overall. Recent studies have shown a renewed interest in neutrophilic biology. One of the most interesting recent discoveries was that the neutrophilic population is not homogeneous. Neutrophilic polarization leads to divergent phenotypes (e.g., a pro- and antitumor profile) that are dynamic subpopulations with distinct phenotypical and functional abilities. Moreover, there is emerging evidence indicating that neutrophils expressing CD64 favor systemic inflammation during ENL. In the present review, neutrophilic involvement in leprosy is discussed with a particular focus on ENL and the potential of neutrophils as clinical biomarkers and therapeutic targets.

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Erythema multiforme in leprosy

Cited by 11 publications

References 24 publications

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Neutrophil extracellular traps contribute to the pathogenesis of leprosy type 2 reactions

Neutrophils in Leprosy

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