Steroid Premedication Might Protect From Postoperative Erythema Nodosum Leprosum in Leprosy: A Case Report.

Leprosy is known for its diverse pathophysiologic involvement and resulting multisystemic manifestation and morbidities. Despite global efforts to eliminate this public health illness, it is still prevalent in some Asian and European countries. Perioperative management of a leprosy patient is challenging owing to the indirect and direct involvement of the airway, respiratory, and cardiac systems; treatment-related side-effects involving the hepato-renal systems affecting the anesthesia techniques and drugs pharmacokinetic and pharmacodynamics. While anaesthesiologists are aware of such happenings and often tailor the anesthesia management for the concerning issues, immunological aspects of the disease and drug-related adverse events are less enquired about, such as type-2 lepra reaction, i.e., erythema nodosum leprosum (ENL), etc. Further, data on perioperative ENL management and prevention are still being determined. We report one case of a 52-year-old female who underwent gynecology surgery and developed ENL on the third postoperative day, which was managed using Steroids. Unfortunately, the patient had a surgical site infection, which required another surgery within the month, while the patient was still under the steroid successfully without any adverse events. Although a single case cannot provide causation or association, the case is presented to highlight the probable preventive action of steroids on the occurrence of postoperative ENL, where surgical stress is considered a risk factor.

Childhood Leprosy in Postelimination Era: A Clinico-Epidemiological Prospective Observational Study from India.

Background: Although India has achieved national level elimination of leprosy in December 2005, it reports highest number of leprosy cases in the world. Incidence of leprosy amongst young children indicates active foci of transmission in the community, making it a robust epidemiological indicator to assess the progress of leprosy control programs. Aim and Objectives: To study prevalence, clinical spectrum, patterns of extracutaneous involvement, reactions, relapse, demographic profile, anthropometric characteristics of children with leprosy. Materials and Methods: A prospective observational study of children younger than 14 years old with clinical signs and symptoms of leprosy, including cases diagnosed from October 2018 through September 2020 at a tertiary hospital in Eastern India. Results: Childhood leprosy constituted of 10.4% of total new leprosy cases. Mean age observed was 11.14 ± 1.5 years. 30.4% were females and 69.6% were males. The mean average duration was 9.95 ± 12.3 months. 82.1% of children belong to below poverty line (BPL) status. 19.6% of children had malnourishment/stunting/thinness, and 55.4% had anemia. 84.1% presented with skin discoloration. The most common site affected was upper limb (57.1%). 29.2% had contact history. A single case of relapse (1.7%) was reported. Most common peripheral nerve involved was ulnar nerve (67.8%). 8.9% of children had type 1 lepra reaction, and 1.7% had type 2 lepra reaction. 73.21% presented with borderline tuberculoid clinical type of leprosy. 19.6% showed slit skin smear positivity. Conclusion: Pockets of high endemicity of childhood leprosy as detected in the current study point toward the unmet need of better healthcare facilities along with increased awareness, campaigns, and school surveys for early diagnosis and treatment for the prevention of complications and spread in community.

A Case of Borderline Tuberculoid Leprosy With Type One Lepra Reaction.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, which primarily affects the skin and peripheral nerves. The variants that can be identified include tuberculoid (TT), borderline tuberculoid (BT), mid-borderline (BB), borderline lepromatous (BL), and lepromatous forms (LL). Type one lepra reactions are delayed hypersensitivity reactions that are often observed in borderline variants due to an unstable immunological response. They can exacerbate skin lesions and neuritis, leading to a higher risk of disabilities and deformities. Early detection and management would play a major role in limiting morbidity. Here, we present a case of a 46-year-old male diagnosed with borderline tuberculoid leprosy on multidrug therapy who developed features suggestive of type one lepra reaction. Early recognition of this entity helps in mitigating the risk of permanent nerve damage, disability, deformity, and morbidity.

Systemic manifestation of necrotic erythema nodosum leprosum: A case report of a fatal leprosy.

Necrotic erythema nodosum leprosum (ENL) is an uncommon manifestation of type 2 lepra reaction, encountered in lepromatous and borderline lepromatous cases of leprosy. Necrotic ENL is associated with the involvement of multiple organs, therefore delayed diagnosis and treatment will lead to complications and poor prognosis. The aim of this case report was to report a challenging case of necrotic ENL misdiagnosed with multiple cellulitis since there were no signs of prior leprosy nor had any antimycobacterial treatment. A 45-year-old man was presented to the surgery department of Dr. Zainoel Abidin Hospital, Banda Aceh, Indonesia, with complaints of fever, joint pain, and painful tender skin lesions with ulceration over the trunk, extremities, and ears for one month. The patient was diagnosed clinically with multiple cellulitis and underwent a debridement procedure. Clinical improvement was absent, the patient was then consulted to the dermatology department. Physical examination showed normal vital signs, madarosis, inguinal lymphadenopathy, thickening of nerves, and sensation of numbness in both hands and feet. Laboratory examinations on admission showed leucocytosis, anemia, thrombocytopenia, hypoalbuminemia, hypocalcemia, and elevated creatinine and ureum level. A slit skin smears examination yielded positive acid-fast bacilli (AFB) with a bacteriological index (BI) value of 3+ and morphological index (MI) of 72%. The patient was diagnosed with lepromatous leprosy with necrotic ENL reaction. Intravenous methylprednisolone and cefoperazone-sulbactam were given. Multidrug therapy mulitbacillary (MDT-MB) without dapsone, and ofloxacin 400 mg was initiated. On day 17, the patient had septic shock. The patient became unconscious and experienced death. This case highlights that medical professionals should be aware of the various manifestations of necrotic ENL to correctly diagnose and provide treatment as soon as possible to prevent mortality, especially in leprosy-endemic country, Indonesia.

Comparison of Serum Levels of Interleukin 17 and Interleukin 9 in Leprosy Patients with and without Lepra Reaction: A Cross-Sectional Analytical Study from Central India.

New pathways of host defence have emerged in leprosy, such as T helper (Th) -17, Th-9, T regulatory cells, and other factors like transforming growth factor-beta, etc. Interleukin (IL) 17 produced by Th17 cells has been found to be elevated in lepra reaction, especially type 2 lepra reaction (T2R). Role of IL-9 has not been studied widely in leprosy reactions so far. The study aimed to compare serum levels of IL-17 and IL-9 in leprosy patients with and without lepra reaction. This was a cross-sectional analytical study including untreated adult leprosy patients with and without lepra reaction. A total of 65 patients were included in the study with 30 leprosy patients without reaction and 35 with lepra reaction. Serum levels of IL-17 and IL-9 were measured in these patients using direct enzyme-linked immunosorbent assay and were compared. Borderline tuberculoid (BT) leprosy with type 1 and Lepromatous (LL) leprosy with T2R patients showed significantly higher levels of IL-17 than BT and LL leprosy patients without lepra reaction, respectively. LL patients with T2R showed significantly lower levels of IL-9 than lepromatous cases without reaction. IL-9 levels were higher in BT patients with T1R as compared to BT patients without reaction but the difference was not significant. We found evidence in support of role of IL-17 in the pathogenesis of T2R, which might serve as useful serum markers for the same. IL-17 might have a role in BT leprosy with T1R. IL-9 seems to have a protective role in T2R as opposed to IL-17, working in synergism with Th1 cytokines.

A Young Female With Borderline Lepromatous Leprosy and Tuberculous Lymphadenitis: A Rare Coinfection.

In Bangladesh, tuberculosis and leprosy are endemic mycobacterial diseases; however, co-infection is rarely seen. Our patient had a high-grade fever, symmetrical polyarthritis, polymorphous erythematous lesions, widespread lymphadenopathy, peripheral neuropathy, bilaterally thickened ulnar nerves, and claw hands. A lymph node biopsy revealed tuberculosis having acid-fast bacilli with caseating epithelioid histiocytic granuloma. Cutaneous lesions and sural nerve biopsies indicated borderline lepromatous leprosy. Fite-Faraco stain showed the presence of lepra bacilli in the biopsied sural nerve. Mantoux test showed 15 mm induration in 72 hours. Nerve conduction study (NCS) showed severe sensory-motor polyneuropathy (axonal) of all four limbs. Prednisolone and thalidomide for severe type-2 lepra response and category-01 antituberculosis medication and multidrug therapy for multibacillary leprosy improved the patient's condition. Bacille Calmette-Guérin (BCG) vaccination in the community might protect against tuberculosis and leprosy, thus reducing such coinfection. However, reduced cell-mediated immunity might promote latent tuberculosis reactivation or super-infection in individuals with multi-bacilli illnesses.

Cutaneous ulceration as a presenting feature of type 1 lepra reaction: A case report.

Reactions in leprosy represent sudden shift in the immunological response and are seen in 11-25% of affected patients. It can be seen before, during or after the completion of multidrug therapy (MDT).1 Two types of reactions are recognized; Type 1 reaction (T1R), seen in borderline leprosy, affecting mainly skin and nerves; type 2 reaction (T2R) or erythema nodosum leprosum (ENL), seen in lepromatous leprosy, characterized by systemic features in addition to cutaneous lesions. Trophic ulcers and ulcerating ENL are well known entities while cutaneous ulceration in T1R is extremely rare; we describe an immune-competent woman with cutaneous ulceration as a presenting feature to highlight the need to recognize this entity at the earliest opportunity.

A case of recurrent steroid-dependent severe type 2 lepra reaction treated successfully with colchicine.

Type 2 lepra reaction (or erythema nodosum leprosum [ENL]) is an immune complex-mediated reaction which has been reported to occur for up to 8 years after successful completion of multibacillary multidrug therapy. The management of ENL is a challenge with the patient often becoming steroid dependent; predisposing to recurrent episodes in 39%-77% of cases on attempted steroid withdrawal. Here, we present a case of recalcitrant recurrent type 2 reaction which responded promptly to treatment with colchicine.

Cyclooxygenase 2 and vascular endothelial growth factor-potential targets to manage lepra reactions: A case-control study.

Reactions in leprosy have an immune mediated pathogenesis. While type 1 reactions are delayed hypersensitivity phenomenon, type 2 reactions are immune complex mediated. Key molecules which mediate the immune insult in lepra reactions require evaluation in order to tailor their therapy and prevent disability. The objective of the study was to evaluate expressions of Cyclooxygenase 2 and Vascular Endothelial Growth Factor in skin biopsies from leprosy patients and correlate their expression with presence of either type 1 or type 2 lepra reactions. This was a case control study. Cyclooxygenase 2 and Vascular Endothelial Growth Factor expression in dermal macrophages and vascular endothelium was assessed immunohistochemically. Biopsies from patients with Non-reactive leprosy and healthy controls were used for comparison. SPSS software was used for statistical analysis. A total of 147 skin biopsies were evaluated, including 18 with Type 1 reaction, 39 Type 2 reaction, 81 non-reactive leprosy and 9 healthy controls. Both Cyclooxygenase 2 and Vascular Endothelial Growth Factor expression were significantly higher in type 1 followed by type 2 reaction as compared to controls. These results may guide us regarding use of Cyclooxygenase 2 and Vascular Endothelial Growth Factor inhibitor drugs which may be a major step in treating reactive leprosy patients and preventing nerve damage and disability.

Target/therapies for chronic recurrent erythema nodosum leprosum.

A Type 2 lepra reaction or erythema nodosum leprosum is an anticipated complication in the lepromatous spectrum of leprosy cases. It is an example of an immune complex-mediated complement activated disease (Type III hypersensitivity reaction). Hence, we tried to target the inflammatory mediators and the mental stressors for the possible management strategies.

What is New in the Pathogenesis and Management of Erythema Nodosum Leprosum.

Erythema nodosum leprosum (ENL) is a manifestation of type II lepra reaction, seen in lepromatous or borderline lepromatous leprosy. Although it is a common reaction encountered in clinical practice, there are an increasingly large number of newer updates in the pathophysiology and management of this condition. The treatment options have expanded far beyond just thalidomide and steroids and now extends to TNF-α inhibitors, thalidomide analogs, tenidap, cyclosporine A, plasma exchange, and even IVIG amongst others. These updates and the current knowledge of ENL are summarized in this review.

Role of Histopathology in Predicting type 1 Lepra Reaction in Borderline Tuberculoid Leprosy.

CONTEXT: Lepra reactions if not managed promptly are an important cause of sudden onset nerve palsy and disability due to leprosy. AIM: To evaluate the usefulness of histology in predicting type 1 lepra reaction. SETTING AND DESIGN: After obtaining clearance from institutional research and ethics committees, all histologically proven borderline tuberculoid patients diagnosed at our center from 1.8.2016 to 31.7.2018 were included in this retrospective cross-sectional study. METHOD: Clinical details were collected from patient records. The pathologist who was blinded to clinical evidence of type 1 lepra reaction at the time of biopsy re-evaluated the histopathology slides for evidence of type 1 reaction. The data of individual patient was analyzed to identify those who had a type 1 reaction at the time of the biopsy or who developed a lepra reaction during follow up. STATISTICAL ANALYSIS USED: Association between histological evidence of type 1 reaction and clinical manifestation of the same subsequently, was assessed using Pearson's Chi square test. RESULTS: Study group comprised of 22 females and 18 males. Clinicohistological concordance was noted in 27 patients (67.5%). Subclinical type 1 reaction was documented in 11 patients (27.5%) based on histopathology evaluation. Five (45.5%) of these 11 patients subsequently developed clinical features of type 1 reaction. This was found to be statistically significant (P value 0.02). LIMITATIONS: Main limitation was the small sample size. CONCLUSIONS: Histology could serve as a useful tool in predicting future type 1 lepra reaction.

Lazarine leprosy: A unique phenomenon of leprosy.

Leprosy, a chronic granulomatous disease, has been known since ages but even today continues to baffle the clinicians with a wide spectrum of clinical, histopathological, and immunological characteristics. Leprosy reactions are mainly of two types, namely, Type 1 and Type 2. In Type 1 leprosy reaction, the preexisting lesions become erythematous, edematous, and rarely ulcerate. Ulcerating Type 1 reaction is called lazarine leprosy. Ulcerations may occur in borderline tuberculoid (BT) pole or borderline lepromatous pole but more common in BT pole. In this postelimination era of leprosy, we report an interesting case report of BT Hansen's disease with Type 1 lepra reaction with ulceration, namely, lazarine leprosy.

Leprosy: A rare case of infectious peripheral neuropathy in the United States.

Peripheral neuropathy can be the initial presentation of leprosy. Diagnosis can be challenging unless skin manifestations are recognized. Skin biopsy and Fite staining are the keys to the diagnosis. It is important to treat coexisting Lepra reactions, peripheral neuropathy and side effects of the therapeutic agents. This is a complex clinical course of a patient with lepromatous leprosy.

Hansen's disease with lepra reaction presenting with IgA dominant infection related glomerulonephritis.

Various renal abnormalities in leprosy have been described largely in literature but the occurrence of IgA dominant infection related glomerulonephritis in leprosy with type 2 lepra reaction has not been reported so far. We present here a 60-year-old man with a history of leprosy in the past admitted with type 2 lepra reaction, rapidly progressive glomerulonephritis with severe renal failure requiring dialysis and diagnosed to have IgA dominant infection related glomerulonephritis.

A dissimulate presentation of histoid Hansen's disease in the form of erythema nodosum leprosum.

Hansen's disease is a chronic infectious granulomatous disease with varied clinical presentation. Histoid Hansen's disease is an important emerging lepromatous subset of Hansen's disease known to mimic varied dermatoses. Occurrence of reactions, especially erythema nodosum leprosum (ENL), is rare in this form of leprosy. We report a case of Histoid Hansen's disease with initial presentation of ENL while undergoing management for infertility.

Two atypical presentations of lepra reactions.

Leprosy or Hansen's disease is a chronic infectious granulomatous disease with varied presentation, especially in the setting of lepra reactions. We report two such atypical presentations each of Type I and Type II Lepra reactions; the first being an elderly male presenting with fever, while the second case being of a young boy being evaluated for cervical lymphadenitis.

Guillain-Barre Syndrome: A Rare Presentation of Borderline Tuberculoid Leprosy with Type 1 Lepra Reaction.

Guillain-Barre syndrome (GBS) is an autoimmune polyradiculoneuropathy usually preceded by respiratory tract or gastrointestinal infection. The pathogenesis in GBS is based on molecular mimicry mechanism. Hansen's disease is common in India and is the most common infectious cause of neuropathy. We describe a 42-year-old man who was being treated for borderline tuberculoid leprosy and developed Type 1 lepra reaction followed by GBS and responded to plasmapheresis. Lepra reaction may lead to exposure of neural antigens, resulting in autoimmune mechanism and demyelination of peripheral nerves.

Serum cytokines in erythema nodosum leprosum versus non-reactional leprosy: A systematic review and meta-analysis

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Erythema nodosum leprosum is an immune-mediated complication of leprosy whose underlying mechanism has not yet been fully elucidated, making management difficult.</p><p style="text-align: justify;"><strong>OBJECTIVES:</strong> To determine the serum cytokine profile of ENL compared to non-reactional leprosy states.</p><p style="text-align: justify;"><strong>METHODS:</strong> An open literature search was performed using MEDLINE, Cochrane Library, TRIP and HERDIN electronic databases using the keywords ("cytokines" or "inflammatory mediators") and ("erythema nodosum leprosum" or "ENL") and ("leprosy" or "lepra"). Studies were selected by two independent review authors. Risk of bias was assessed using the Newcastle-Ottawa Scale and statistical analysis was performed using RevMan 5.3 software.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Eight cross-sectional studies with 197 participants were included. Meta-analysis showed that both serum IL-17 and serum IFN-? were significantly decreased (Z 2.39, p = 0.02 and Z 2.74, p = 0.01, respectively) in ENL compared to non-reactional states. However, for IL-1?, IL-6, IL-10, IL-22, TNF-? and TGF-?, no significant differences were found between the two groups.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> ENL appears to be an exacerbation of the Th2 cytokine response seen in the lepromatous pole of leprosy. However, despite pooling of data, sample sizes remain small resulting in significant heterogeneity. Future studies involving large sample sizes and investigating a wider range of cytokines are encouraged.</p>