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BACKGROUND Leprosy, also known as Hansen's disease, is a neglected tropical disease with low prevalence in the United States. The disease's long incubation period can cause delayed presentation, and most affected individuals have a history of travel or work in leprosy-endemic regions. The immune response to Mycobacterium leprae determines the clinical characteristics of leprosy, with tuberculoid leprosy being characterized by well-defined granulomas and involvement of peripheral nerves. The recommended treatment is a combination of dapsone and rifampin for 12 months. CASE REPORT A 78-year-old man with a history of extensive travel to Africa and Asia 50 years ago, presented with a non-tender, non-pruritic, and hypopigmented skin lesion on his left knee. Biopsy results confirmed granulomatous inflammation and the presence of Mycobacterium leprae, leading to a diagnosis of tuberculoid/paucibacillary leprosy. The patient received dapsone and rifampin treatment, which resulted in symptom improvement. CONCLUSIONS The patient's long incubation period of 50 years between exposure and symptom onset is remarkable and possibly one of the longest reported for tuberculoid leprosy. It emphasizes the importance of considering leprosy in cases with an extensive travel history and long incubation periods. Our patient's case presented contradictory staining results, suggesting potential sampling variation or a rare mixed leprosy form. Based on his clinical findings, he was diagnosed with tuberculoid leprosy. Early diagnosis and treatment are crucial to prevent irreversible nerve damage and improve patient outcomes. Healthcare providers should be vigilant in acquiring a detailed travel history to facilitate early diagnosis and appropriate management of leprosy cases.

Introduction: Pure Neural Leprosy (PNL) is a form of this long time known disease that affects only the peripheral nervous system. Since it is a rare form of the disease, its pathophisiology is still poorly understood., Objective: Describe the cytokines profile in patients with PNL., Methods: 30 Patients diagnosed with PNL in the Souza Araujo Outpatient Clinic and with cytokines evaluated were selected. They were evaluated by neurologists and diagnosed after a nerve biopsy. Serum levels of IL-1 β, IL-6, IL-10, IL-17, TNF, CCL-2/MCP-1, IFN-ϒ, CXCL-10/IP-10 and TGF-β were evaluates at the moment of the diagnosis., Results: Neural thickening was a common clinical finding in this groups of patients. Small and medium sensitive fibers signs and symptoms were present in 92% of the patients and motor involvement in 53%. 43% of patients presented neuropathic pain and no one had neuritis TGF-beta, IL-17, CCl-2 and IP-10. CCL-2 levels were associated with demyelinating patters and IP-10 and IL-1o were associated with axonal patterns at NCS., Discussion: PNL patients' cytokine profile appears to be different of other clinical forms of leprosy, with the presence of cytokines described in both tuberculoid and lepromatous leprosy. High levels of CCl-2 may be related to the presence of silent neuritis as well as the presence of IL-10. PNL is unique a form of leprosy, therefore, understanding its immunological profiles essential to better understand the disease itself., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Pitta, Angst, Pinheiro, Vieira, Spitz, Andrade, Carvalho, Hacker, Sarno and Jardim.)

Disability in leprosy is a direct consequence of damage to the peripheral nervous system which is usually worse in patients with no skin manifestations, an underdiagnosed subtype of leprosy known as primary neural leprosy. We evaluated clinical, neurophysiological and laboratory findings of 164 patients with definite and probable primary neural leprosy diagnoses. To better understand the disease progression and to improve primary neural leprosy clinical recognition we compared the characteristics of patients with short (≤12 months) and long (>12 months) disease duration. Positive and negative symptoms mediated by small-fibres were frequent at presentation (∼95%), and symptoms tend to manifest first in the upper limbs (∼68%). There is a consistent phenotypic variability between the aforementioned groups. Deep sensory modalities were spared in patients evaluated within the first 12 months of the disease, and were only affected in patients with longer disease duration (∼12%). Deep tendon reflexes abnormalities were most frequent in patients with longer disease duration (P < 0.001), as well as motor deficits (P = 0.002). Damage to large fibres (sensory and motor) is a latter event in primary neural leprosy. Grade-2 disability and nerve thickening was also more frequent in cases with long disease duration (P < 0.001). Primary neural leprosy progresses over time and there is a marked difference in clinical phenotype between patients with short and long disease duration. Patients assessed within the first 12 months of symptom onset had a non-length-dependent predominant small-fibre sensory neuropathy, whilst patients with chronic disease presented an asymmetrical all diameter sensory-motor neuropathy and patchily decreased/absent deep tendon reflexes., (© The Author(s) (2021). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Hansen's disease or leprosy is a chronic infectious disease caused by Mycobacterium leprae, mainly affecting the skin and peripheral nerves. In recent years, a better control of the disease has been achieved globally. However, in Chile the incidence of leprosy has continued to increase. We present a case of a 40-year-old patient Haitian nationality with lesions and histopathology compatible with tuberculoid leprosy. We discuss the importance of education on less prevalent diseases in Chile, in conjunction with a review of the pathophysiology, clinic, and classification of Hansen's disease.

Introduction: Pure Neural Leprosy (PNL) is a rare clinical form of leprosy in which patients do not present with the classical skin lesions but have a high burden of the disability associated with the disease. Clinical characteristics and follow up of patients in PNL are still poorly described in the literature., Objective: This paper aims to describe the clinical, electrophysiological and histopathological characteristics of PNL patients, as well as their evolution after multidrug therapy (MDT)., Methods: Fifty-two PNL patients were selected. Clinical, nerve conduction studies (NCS), histopathological and anti-PGL-1serology were evaluated. Patients were also assessed monthly during the MDT. At the end of the MDT, all of the patients had a new neurological examination and 44 were submitted to another NCS., Results: Paresthesia was the complaint most frequently reported by patients, and in the neurological examination the most common pattern observed was impairment in sensory and motor examination and a mononeuropathy multiplex. Painful nerve enlargement, a classical symptom of leprosy neuropathy, was observed in a minority of patients and in the motor NCS axonal injuries, alone or in combination with demyelinating features, were the most commonly observed. 88% of the patients did not present any leprosy reaction during MDT. There was no statistically significant difference between the neurological examinations, nor the NCS pattern, performed before and after the MDT., Discussion: The classical hallmarks of leprosy neuropathy are not always present in PNL making the diagnosis even more challenging. Nerve biopsy is an important tool for PNL diagnosis as it may guide therapeutic decisions. This paper highlights unique characteristics of PNL in the spectrum of leprosy in an attempt to facilitate the diagnosis and management of these patients., Competing Interests: The authors have declared that no competing interests exist.

Objective: To review the literature and to report a clinical case with initial suspicion of pure neural leprosy and final diagnosis of amyloid neuropathy., Methods: The study was conducted in two stages. In stage one, a systematic literature review was carried out, with searches performed in the PubMed, Medline, and Lilacs databases, as well as in the leprosy sectoral library of the Virtual Health Library, using the following descriptors: neuritic leprosy, pure neural leprosy, primary neural leprosy, pure neuritic leprosy, amyloid polyneuropathy, amyloid neuropathies, and amyloid polyneuropathy. The search was carried out on May 28, 2020. Clinical trials, cohort studies, cross-sectional studies, clinical cases, and case studies published in Portuguese, English or Spanish between 2010 and 2020 were included. Stage two reports a case with initial suspicion of pure neural leprosy. Laboratory tests, electroneuromyography, ultrasound, and biopsy of the sural nerve were requested., Results: Twenty-three scientific texts were included. No publications were found that contained both topics together. The challenging diagnosis of pure neural leprosy and the possibility of using auxiliary resources in diagnosis were the most emphasized themes in the studies. In the clinical case, the patient's electroneuromyography showed sensitive and motor polyneuropathy of the lower limbs, which was predominantly sensory and axonal, symmetrical, of moderate intensity, and the mixed type (axonal-demyelinating). Ultrasonography of the sural nerve revealed changes in the contour of the deep fibular nerves; biopsy of the sural nerve showed an accumulation of amorphous eosinophilic material in the nerve path, and Congo red stain showed apple-green birefringence of the deposit under polarized light. The final diagnosis was amyloid neuropathy., Conclusions: The final clinical diagnosis was amyloid neuropathy. The diagnosis of pure neural leprosy in endemic areas in Brasil is still a challenge for the health system.

Background: The early recognition of neural impairment in leprosy, especially in primary neural forms, represents a challenge in clinical practice and a peripheral nerve biopsy may be required for diagnostic confirmation. This study aims to characterize the epidemiological, clinical, electroneuromyographic, laboratory and histopathological aspects of patients undergoing peripheral nerve biopsy during investigation of primary neural cases in leprosy., Methods: A total of 104 patients with peripheral neuropathy who were referred to a national reference centre for leprosy were biopsied from 2014 to 2018. All cases underwent clinical, laboratory, histopathological and electroneuromyographic evaluations., Results: Of 104 biopsied patients, leprosy was confirmed in 89.4% (93/104). The biopsied nerves were the ulnar (67.8% [63/93]), superficial fibular (21.5% [20/93]), sural (8.6% [8/93]), radial (1.1% [1/93]) and deep fibular (1.1% [1/93]). Twenty-nine percent (27/93) presented histopathological abnormalities and 4.4% (4/93) presented acid-fast bacilli. Nerve and superjacent skin quantitative polymerase chain reaction were positive in 49.5% (46/93) and 24.8% (23/93) of cases, respectively. Patients with multiple mononeuropathy had a higher frequency of histopathological abnormalities (p=0.0077)., Conclusions: This study reinforces peripheral nerve biopsy's role as an important tool in the investigation of primary neural cases, contributing to the early diagnosis and also reducing diagnostic errors and the need for empirical treatment., (© The Author(s) 2020. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene.)

Identification of Mycobacterium leprae DNA by polymerase chain reaction (PCR) is a reliable and an affordable method to confirm leprosy. DNA from 87 nerve samples (61 from paraffin blocks and 26 fresh samples) was extracted. Mycobacterium leprae DNA was amplified by PCR from 80/87 (92%) specimens. Patients were seen over a period of 11 years (2007-2019), and leprosy was diagnosed based on clinical and characteristic histopathology findings. The clinical diagnostic possibilities were as follows: leprous neuropathy in 73/80 (91.3%), mononeuritis multiplex of unknown etiology in four (5.0%), vasculitic neuropathy in two (2.5%), and distal symmetric sensory motor neuropathy in one (1.3%). The biopsied nerves were as follows: superficial radial = 34 (42.6%), dorsal cutaneous branch of ulnar = 19 (23.8%), sural = 18 (22.5%), and superficial peroneal = 9 (11.3%), and corresponding neurological deficits were recorded in 77 (96.3%) cases. The histopathological diagnoses in total group were as follows: (borderline tuberculoid (BT) = 52, tuberculoid (TT) = 8, borderline lepromatous (BL) = 8, borderline borderline (BB) = 3, nonspecific inflammation = 3, healed/fibrosed = 4, and axonopathy = 2). Acid fast bacilli (AFB) was demonstrated in 11 (13.7%) samples. For comparison, 31 clinically and histopathologically defined non-leprous disease control nerves (inherited neuropathy = 20, vasculitis = 8, and nutritional neuropathy = 3) subjected to PCR were negative for M. leprae DNA. In most instances, there are multiple thickened peripheral nerves in suspected cases of leprosy, but neurological deficits pertaining to the thickened nerve are not as widespread. The current findings emphasize the importance of selecting the most appropriate nerve for biopsy to obtain a positive PCR result. We infer that clinical, histopathological, and PCR tests complement each other to help achieve a definitive diagnosis of leprosy particularly in pure neuritic leprosy and in leprous neuropathy with negative skin smears/biopsy.

Leprosy is a chronic infectious disease that presents with varied manifestations. Pure neuritic leprosy is one of the rarest forms of the disease which is characterized by nerve involvement without the characteristic cutaneous stigmata. Eleven year old, healthy male presented with progressively increasing painful swelling at the medial aspect of the arm near to the right elbow joint with difficulty in extending right ring and little fingers at interphalangeal joint and numbness in the same region for last 1 year with no cutaneous abnormalities. Physical examination revealed 6x3 cm firm, tender lesion 3 cm proximal to the right elbow joint with positive tinel's sign, without signs of inflammation, along with characteristic claw hand deformity of right hand and atrophy of hypothenar and interossei muscle. Electro-diagnostic testing revealed findings consistent with a right ulnar axonal neuropathy above the elbow. Magnetic resonance imaging revealed well defined heterogeneously hyper intense linear lesion along the course of thickened ulnar nerve in the distal arm extending posterior to the medial condyle. It also showed an oval shaped lesion (2.1x1.0 cm) arising from the same segment of the nerve, without any bony or muscular involvement of that area. The patient underwent surgical exploration and ulnar nerve decompression with biopsy. Pathology revealed necrotizing granulomatous inflammatory acid fast bacilli stain negative lesion, which was histologically consistent with caseous abscess caused by tuberculoid leprosy, pathognomonic for Hansen's disease. He has been started on antibiotic therapy and is referred to leprosy center for further course of management. Pure neuritic leprosy, though rare, should be considered as differential diagnosis in cases presenting with peripheral neuropathy at leprosy-endemic areas. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

This was a 50-year-old woman with a selling activity living in Lomé who came for a consultation in March 2016 for a facial flushing that had been going on for 2 months without pain or pruritus. On examination, there was a single, erythemato-squamous closet of the right hemiface. There was no infiltration of the right ear. There was moderate cutaneous heat compared to the left hemiface which was without any lesion. Examination of nails, hair, palms and plants was normal. There was no hypertrophy of the peripheral nerves (superficial cervical plexus, ulnar, median). The face was not fixed. Complementary examinations noted a normal blood count and negative HIV status. Histology performed on a biopsy fragment concluded tuberculoid leprosy. The patient was first put on WHO multidrug therapy during 6 months. But one month after stopping this treatment, the lesions resumed. She was referred to a multibacillary leprosy protocol during one year. She had been seen 4 months after stopping treatment, without recurrence. It is important not to ignore leprosy in case of atypical erythema of the face even in the absence of other evocative signs and to perform a biopsy to the slightest doubt., Competing Interests: The authors have no conflicts of interest to declare

Mycobacterium leprae causes leprosy, a dermatoneurological disease which affects the skin and peripheral nerves. One of several cellular structures affected during M. leprae infection is the endoplasmic reticulum (ER). Infection by microorganisms can result in ER stress and lead to the accumulation of unfolded or poorly folded proteins. To restore homeostasis in the cell, the cell induces a series of signaling cascades known as the unfolded protein response called UPR (unfolded protein response). The present work is aimed at investigating the in situ expression of these markers in cutaneous lesions of clinical forms of leprosy and establish possible correlation expression patterns and types of lesion. A total of 43 samples from leprosy patients were analyzed by immunohistochemistry with monoclonal antibodies against GRP78/BiP, PERK, IRE1 α , and ATF6. A statistically significant difference between the indeterminate, tuberculoid, and lepromatous clinical forms was detected, with high expression of GRP78/BiP, PERK, IRE1 α , and ATF6 in tuberculoid forms (TT) when compared to lepromatous leprosy (LL) and indeterminate (I) leprosy. These results represent the first evidence of ER stress in samples of skin lesions from leprosy patients. We believe that they will provide better understanding of the complex pathogenesis of the disease and facilitate further characterization of the cascade of molecular events elicited during infection.

Pure neuritic leprosy (PNL) accounts for 5% to 10% of leprosy patients who usually present with asymmetrical neuropathy in the absence of lepra bacilli on slit-skin smears. However, nerve biopsies in PNL lack appropriate categorization in current immunologic terms. We aimed to classify nerve biopsies according to the immune spectrum of leprosy and assess the role of histologic classification of nerve biopsies in treating PNL. Patients from two tertiary care referral centres were enrolled in this incident case study. Patients presenting with mononeuropathy and multiple mononeuropathies presumably with leprosy, without skin lesions, underwent nerve biopsy and slit-skin smear examination. Amongst 78 patients with mononeuropathy, 38 were diagnosed with leprosy on nerve biopsy. Leprosy was classified as tuberculoid in 16, lepromatous in 5 and borderline in 17 patients. Lepra bacilli were present in 15 biopsies. On comparing histologic subtypes with number of nerves involved clinically, a significant number of cases with single nerve involvement showed multibacillary (BB, BL or LL) histology and vice versa. Nerve biopsy helps in diagnosing patients presenting with PNL and aids in classifying it to customize the treatment for best results. Current treatment recommendations for PNL from WHO and National Leprosy Eradication Program are based on clinical assessment only, which are likely to result in inconsistent treatment and possibly relapse in cases where histomorphology shows disparity. Inclusion of nerve biopsy to guide therapy in patients with PNL is suggested., (© 2018 Peripheral Nerve Society.)

Pure neural leprosy, defined as a peripheral neuropathy in which the patient has no skin lesions, is difficult to diagnose. Its verification by bacteriological index and histopathology is not possible in the majority of the patients.We describe four cases of pure neural leprosy diagnosed by clinical criteria. The clinical outcome of three of the patients after specific treatment was satisfactory, while the other one developed progressive neural damage despite the therapy. All patients were treated in a specialized center for the management and control of Hansen's disease in the municipality of Contratación, Santander, Colombia.

Background: More than 200 000 new cases of leprosy are detected worldwide annually. Physicians commonly have difficulty in differentiating tuberculoid form of leprosy (TL) from sarcoidosis' cutaneous manifestation., Methods: Skin biopsies of 33 patients with TL and 24 with sarcoidosis were reviewed on hematoxylin and eosin- and Gomori-stained sections, in order to find reliable criteria for distinguishing one disease from another., Results: Nine of the 24 features analyzed presented significant predictive value for diagnosis (P < .05). Predominance of tuberculoid granulomas in adnexal and neural distribution, and granulomas replacing the nerves localized within sweat gland glomeruli were predictive to TL diagnosis. For sarcoidosis, dermal fibrosis, back-to-back distribution of the granulomas, presence of atypical giant cells and plasma cells, greater number of conventional giant cells, and spared nerves beside the granuloma were predictive criteria. The median surface density of reticulin fibers was significantly higher in sarcoidosis (3.44) than in TL (2.99). Nonetheless, using logistic regression, this variable did not discriminate between the diseases (P = .096)., Conclusions: Isolated histological features are not fully predictive to differentiate the 2 diseases. However, those with statistical value can assist this distinction in diagnostic practice. Although the results of the analysis of the reticulin fibers density did not tell apart TL from sarcoidosis, they corroborate the idea of fiber fragmentation within tuberculoid leprosy granulomas, reiterating the importance of morphometry in the histological examination., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

A 34-year-old Indian man presented to an orthopaedician with gradually progressive hypoesthesia affecting his right lower limb and an ipsilateral common peroneal nerve swelling around the knee. The nerve swelling was diagnosed as a peripheral nerve sheath tumour based on MRI findings and was excised, only to be revealed as leprous nerve abscess on histopathology later. The patient developed right foot drop as a result of common peroneal nerve biopsy. This case presents several learning points in the diagnosis of pure neural leprosy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Leprosy neuropathy is considered the most common peripheral neuropathy of infectious etiology worldwide, representing a public health problem. Clinical diagnosis of primary neural leprosy (PNL) is challenging, since no skin lesions are found and the slit skin smear bacilloscopy is negative. However, there are still controversial concepts regarding the primary-neural versus pure-neural leprosy definition, which will be explored by using multiple clinical-laboratory analyses in this study., Methodology/principal Findings: Seventy patients diagnosed with primary neural leprosy from 2014 to 2016 underwent clinical, laboratorial and neurophysiological evaluation. All patients presented an asymmetric neural impairment, with nerve thickening in 58.6%. Electroneuromyography showed a pattern of mononeuropathy in 51.4%. Positivity for ELISA anti-PGL1 was 52.9%, while the qPCR of slit skin smear was 78.6%. The qPCR of nerve biopsies was positive in 60.8%. Patients with multiple mononeuropathy patterns showed lower levels of anti-PGL-1 (p = 0.0006), and higher frequency of neural thickening (p = 0.0008) and sensory symptoms (p = 0.01) than those with mononeuropathy., Conclusions/significance: PNL is not a synonym of pure neural leprosy, as this condition may include a generalized immune response and also a skin involvement, documented by molecular findings. Immunological, molecular, and neurophysiological tools must be implemented for diagnosing primary neural leprosy to achieve effective treatment and reduction of its resultant disabilities that still represent a public health problem in several developing nations. Finally, we propose a algorithm and recommendations for the diagnosis of primary neural leprosy based on the combination of the three clinical-laboratorial tools.

Leprosy is a chronic granulomatous infectious multisystem disease that may present with protean manifestations. It mimics many systemic and dermatological disorders. Here we report a case in which an elderly female presented with malar rash, intermittent fever, and arthralgia. Her diagnosis was significantly delayed due to a close clinical resemblance to systemic lupus erythematosus. It is important to be aware of such manifestations of leprosy and improve awareness of it in clinicians to avoid misdiagnosis and delay in treatment.

Leprosy is a chronic infectious disease caused by Mycobacterium (M.) leprae. Worldwide, 210,758 new cases were diagnosed in 2015. The highest incidence is found in India, Brazil, and Indonesia. While the exact route of transmission remains unknown, nasal droplet infection is thought to be most likely. The pathogen primarily affects the skin and peripheral nervous system. The disease course is determined by individual host immunity. Clinically, multibacillary lepromatous variants are distinguished from paucibacillary tuberculoid forms. Apart from the various characteristic skin lesions, the condition is marked by damage to the peripheral nervous system. Advanced disease is characterized by disfiguring mutilations. Current treatment options are based on WHO recommendations. Early treatment frequently results in complete remission without sequelae. While paucibacillary forms are treated with rifampicin and dapsone for at least six months, multibacillary leprosy is treated for at least twelve months, additionally requiring clofazimine. Leprosy reactions during therapy may considerably aggravate the disease course. Besides individual treatment, WHO-supported preventive measures and strategies play a key role in endemic areas., (© 2017 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)

Immune reconstitution inflammatory syndrome is an inflammatory reaction in HIV-infected patients after initiation of antiretroviral therapy resulting from restored immunity to specific infectious or non-infectious antigens. A 36-year-old male patient on highly active antiretroviral therapy of six months duration, presented with reddish, tender lesions over medial aspect of arm and a single, anaesthetic patch. Tender fluctuant swellings were seen on the medial aspect of left forearm. A few of them had ruptured spontaneously discharging pus. A skin biopsy from the anaesthetic patch showed caseating epitheloid granulomas. A diagnosis of Hansen's disease borderline tuberculoid in type 1 reversal reaction, with formation of nerve abscess due to Immune Reconstitution Inflammatory Syndrome was made. The patient was started on multibacillary multidrug therapy as per WHO guidelines and highly active antiretroviral therapy was continued.

Objective/background: Clinical diagnosis of indeterminate and tuberculoid leprosy is often difficult due to limited and confounding signs and symptoms. In the current study, we evaluated the utility of new multiplex polymerase chain reaction (PCR) using Mycobacterium leprae-specific DNA sequences in the pseudogene regions of ML1545, ML2180, and ML2179 for PCR-based diagnosis of indeterminate leprosy (IND) and leprosy cases across the immunological spectrum. The sensitivity was compared with that of RLEP PCR., Methods: DNA was extracted from paraffin-embedded skin biopsy specimens of 220 leprosy cases, which were divided into IND (41), tuberculoid form (3), borderline tuberculoid (42), midborderline (3), borderline lepromatous (n=59), and lepromatous leprosy (72) cases. PCR positivity of both multiplex and RLEP PCR were compared in all the samples. A decision tree was constructed using the classification and regression trees algorithm to predict the probability of PCR positivity with the new multiplex PCR scheme in various clinical groups of leprosy. Sensitivity of each pseudogene target was determined using real-time PCR assays, and specificity was confirmed by PCR amplification of DNA extracted from three other mycobacterial species and skin biopsies of 44 non-leprosy cases., Results: A multiplex PCR positivity of 75.61% was noted in IND cases when compared to that of 58.54% using RLEP PCR (P < 0.05). Enhanced multiplex PCR positivity was noted across various clinical groups in comparison to RLEP PCR. The decision tree classifier has predicted statistically significant probability for multiplex PCR positivity among RLEP-PCR negative group and clinical groups with a low bacillary load., Conclusion: This new multiplex PCR scheme can support the diagnosis of indeterminate and tuberculoid forms of leprosy with limited clinical manifestations and can be implemented in basic clinical/diagnostic setting that possess conventional PCR facilities.

Introduction: Histopathologic diagnosis of leprosy is difficult when Bacillary Index (BI) is zero and neural involvement are not easily identifiable on routine Hematoxylin and Eosin stain. This study was undertaken to study the role of S-100 immunostaining in demonstrating different patterns of nerve involvement in various types of leprosy., Methods: Thirty one skin biopsies with clinico-histopathologic diagnoses of leprosy over a period of two years were included in the study. Ten cases of non-lepromatous granulomatous dermatoses (including eight cases of lupus vulgaris and two cases of erythema nodosum) were used as controls. Tissue sections from all cases and controls were stained with Hematoxylin and Eosin (H&E) stain, Fite stain and S-100 immunostain. The H&E stained slides were used to study the histopathological features, Fite stained slides for Bacillary Index and S-100 for nerve changes., Results: Neural changes could be demonstrated in the entire spectrum of leprosy using S-100 immunostaining. The most common pattern of nerve destruction in the tuberculoid spectrum was fragmented and infiltrated whereas lepromatous spectrum showed mostly fragmented nerve twigs. Intact nerves were not detected in any of the leprosy cases., Conclusions: S-100 immunostain is a useful auxiliary aid to the routine H&E stain in the diagnosis of leprosy especially tuberculoid spectrum and intermediate leprosy.

Background: Histological similarities between granulomas and granulomatous mycosis fungoides (GMF) may lead to misdiagnoses of sarcoidosis or leprosy., Methods: This report presents four patients with GMF in whom skin biopsies showed perineural and intraneural granulomas that were confused with tuberculoid leprosy granulomas., Results: Patient 1 presented with erythematous plaques and bulky nodules. Biopsy findings suggested cutaneous sarcoidosis. Tumor resection showed granulomatous infiltrate extending to the fascia and skeletal muscle. Clinicopathological correlations permitted a diagnosis of GMF. Patient 2 presented with erythematous plaques. Skin biopsies had indicated sarcoidosis. Resection of a thigh nodule excluded leprosy, and GMF was diagnosed. Patient 3 presented with scaly, hyperpigmented plaques. Biopsy showed diffuse granulomatous inflammation with epithelioid and giant cells, abundant lymphocytes, and some eosinophils, and indicated GMF. Patient 4 presented with pruritic, erythematous plaques. Biopsy of an indurated mammary plaque initially indicated sarcoid granulomatous inflammation. Biopsy review suggested GMF., Conclusions: This study highlights both the diagnosis of GMF, and granulomatous cutaneous nerve injury in GMF and its possible confusion with leprosy granulomas. The histological diagnosis of GMF includes: (i) a granulomatous infiltrate rich in giant cells, emperipolesis, histiocytic cells, and scattered eosinophils, which may reach the fascia and muscle; (ii) the absence of elastic fibers or their phagocytosis by giant cells; and (iii) lymphocytes that may show atypia and epidermotropism. Deep biopsies reveal GMF diagnostic changes and, in conjunction with clinicopathological correlations, exclude a diagnosis of leprosy and support one of GMF, thus facilitating its appropriate management., (© 2016 The International Society of Dermatology.)

Leprosy is a chronic granulomatous infection caused by Mycobacterium leprae with predominant involvement of skin and nerves. We present a 70-year-old man with leprosy whose initial presentation resembled rheumatologic disease, due to leprae reaction. He presented with an 8-week history of worsening neuropathic pain in the right forearm, associated with necrotic skin lesions on his fingers that had ulcerated. Physical examination revealed two tender necrotic ulcers at the tip of the right middle finger and the dorsal aspect of the left middle finger. The patient had right wrist tenosynovitis and right elbow bursitis. Apart from raised inflammatory markers, the investigations for infection, connective tissue disease, vasculitis, thromboembolic disease and malignancy were negative. During the fourth week of hospitalization, we noticed a 2-cm hypoesthetic indurated plaque on the right inner arm. Further examination revealed thickened bilateral ulnar, radial and popliteal nerves. A slit skin smear was negative. Two skin biopsies and a biopsy of the olecranon bursa revealed granulomatous inflammation. He was diagnosed with paucibacillary leprosy with neuritis. He responded well to multidrug therapy and prednisolone; his symptoms resolved over a few weeks. This case illustrates the challenges in diagnosing a case of leprosy with atypical presentation in a non-endemic country., (© 2016 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Leprosy (Hansen's disease) is a chronic contagious granulomatous disease principally affecting the skin and peripheral nervous system, caused by Mycobacterium leprae. In this report, we present a case of autochthonous leprosy in a man from Florida as the first human case reported from this region. Authors believe dermatologists need to be aware of the possibility of autochthonous transmission of leprosy in the Eastern-Southern United States, and should consider leprosy in any patient with atypical skin lesions, even when a history of contact with armadillo is missing.

Hansen's disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansen's disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansen's disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Leprosy and tuberculosis are chronic mycobacterial infections that elicit granulomatous inflammation. Both infections are curable, but granulomatous injury to cutaneous structures, including cutaneous nerves in leprosy, may cause permanent damage. Both diseases are major global concerns: tuberculosis for its high prevalence and mortality, and leprosy for its persistent global presence and high rate of neuropathic disability. Cutaneous manifestations of both leprosy and tuberculosis are frequently subtle and challenging in dermatologic practice and often require a careful travel and social history and a high index of suspicion., (Published by Elsevier Inc.)

Leprosy has been increasingly known to have an enigmatic relationship with human immunodeficiency virus infection. Co-infection may result in atypical manifestations of leprosy. A 45-year old human immunodeficiency virus-positive male; agricultural laborer presented with a swelling over right elbow, right hand deformity, generalized itching and recurrent vesicles overthe perinasal area. Clinical and investigational findings were consistent with mononeuritic type of Hansen's disease with right sided silent ulnar nerve abscess, partial claw hand. CD4+ count of the patientwas 430 cells/cmm. This patient also hadherpes simplex labialis, with HIV-associated pruritus. To the best of our knowledge such an atypical presentation has not been reported earlier.

Background: Leprosy continues to be present in certain regions throughout the world, and the dermatologist plays a central role in its diagnosis. Herein we report a case of tuberculoid leprosy that is atypical in terms of its linear presentation which appears to follow the lines of Blaschko., Patients and Methods: A patient from Mayotte was referred to the neurological department for suspected tuberculoid leprosy. He was presenting a deficiency of the ulnar nerve together with neuronal hypertrophy and cutaneous involvement. Dermatological examination revealed linear hypo-aesthetic hypopigmented lesions on the arm and forearm. The atypical clinical presentation also suggested to us pigmented mosaicism or post-inflammatory pigmentation. The biopsy showed granulomatous epithelial dermatitis with perinervous involvement. Imaging examinations confirmed the presence of neuronal hypertrophy. A diagnosis of linear tuberculoid leprosy was made., Discussion: This case illustrates the need to bear in mind a diagnosis of leprosy in the event of hypopigmented lesions, even where they are linear and of Blaschkoid appearance. Several potential hypotheses may account for this particular topographical pattern., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Linear distribution of multiple subcutaneous nodules or ulcers along the course of lymphatics, classically seen in lymphocutaneous sporotrichosis, has been observed in a number of other infections like localized cutaneous leishmaniasis, cutaneous tuberculous and non tuberculous mycobacterial infections, Pasteurella tularensis, Scopulariopsis blochi, Nocardia brasiliensis, yaws and syphilis. A case of borderline tuberculoid leprosy with multiple cutaneous nodules corresponding to resolving nerve abscesses in a sporotrichoid pattern is being reported.

Leprosy is endemic in Senegal. In 2011, there were 73 new cases reported in Dakar. The circumstances of discovery are often dermatologic or neurologic. Few case reports describe an association with chronic inflammatory colitis, probably fortuitous. We report the case of a 30-year-old woman who had a tuberculoid leprosy revealed by active ulcerative colitis. Treatment according to the WHO protocol of leprosy, combined with corticosteroids and then methotrexate, resulted in healing of the leprosy and remission of the colitis.

We present the case of a native Texan who was diagnosed with tuberculoid leprosy and later developed a cutaneous infection with M. haemophilum following iatrogenic immunosuppression. To our knowledge, there are no such reports of M. haemophilum and M. leprae infection occurring simultaneously in the same host., (© 2014.)

The diagnosis of pure neural leprosy (PNL) is based on clinical and laboratory data, including the histopathology of nerve biopsy specimens and detection of Mycobacterium leprae DNA by polymerase chain reaction (PCR). Given that histopathologic examination and PCR methods may not be sufficient to confirm the diagnosis, immunolabeling of lipoarabinomanan (LAM) and/or phenolic glycolipid 1 (PGL-1) M. leprae wall components was utilized in the present investigation in an attempt to detect any vestigial presence of M. leprae in acid-fast bacilli (AFB) nerve samples. Twenty-three PNL nerve samples (6 AFB and 17 AFBPCR) were cryosectioned and subjected to LAM and PGL-1 immunohistochemical staining by immunoperoxidase. Five nonleprosy nerve samples were used as controls. The 6 AFB samples showed LAM/PGL-1 immunoreactivity. Among the 17 AFB samples, 8 revealed LAM and/or PGL-1 immunoreactivity. In 17 AFBPCR patients, just 7 yielded LAM and/or PGL-1 nerve results. In the PNL cases, the detection of immunolabeled LAM and PGL-1 in the nerve samples would have contributed to an enhanced diagnostic efficiency in the absence of molecular diagnostic facilities.

Leprosy maybe "unmasked" in the context of immune reconstitution inflammatory syndrome and treating dermatologists, particularly in highly endemic areas for Hansen's disease, need to be cognizant to this possibility. It may also reflect emergence of a previously clinically silent infection in the course of immunologic restoration.

Background: The diagnosis of pure neural leprosy (PNL) remained subjective because of over-dependence of clinical expertise and a lack of simple yet reliable diagnostic tool. The criteria for diagnosis, proposed by Jardim et al., are not routinely done by clinicians in developing country as it involves invasive nerve biopsy and sophisticated anti-PGL-1 detection. We conducted a study using fine needle aspiration cytology (FNAC) coupled with Ziehl Neelsen staining (ZN staining) and Multiplex-Polymerase Chain Reaction (PCR) specific for M. leprae for an objective diagnosis of pure neural leprosy (PNL), which may be simpler and yet reliable., Aim: The aim of the study is to couple FNAC with ZN staining and multiplex PCR to diagnose pure neural leprosy patients rapidly, in simpler and yet reliable way., Methods: Thirteen patients of PNL as diagnosed by two independent consultants were included as case, and 5 patients other than PNL were taken as control in the study. Fine needle aspiration was done on the affected nerve, and aspirates were evaluated for cytology, ZN staining and multiplex-PCR., Results: Out of the 13 cases where fine needle aspiration was done, M. leprae could be elicited in the nerve tissue aspirates in 5 cases (38.4%) with the help of conventional acid-fast staining and 11 cases (84.6%) with the help of multiplex PCR. On cytological examination of the aspirates, only 3 (23%) cases showed specific epithelioid cells, whereas 8 (61.5%) cases showed non-specific inflammation, and 2 (15.3%) cases had no inflammatory cells., Conclusion: Our study demonstrates that in the field of laboratory diagnosis of PNL cases, FNAC in combination with ZN staining for acid-fast bacilli (AFB) and Multiplex-PCR can provide a rapid and definitive diagnosis for the majority of PNL cases. FNAC is a less-invasive, outdoor-based and simpler technique than invasive nerve biopsy procedure. Thus, this study may enlighten the future path for easy and reliable diagnosis of PNL.

A 65-year-old unemployed man, originally from Michoacán and currently living in Toluca, state of Mexico, presented for medical consultation for disseminated dermatosis in all body segments. The condition was limited to the head and neck, was bilateral and symmetrical, and was characterized by infiltrated and confluent erythematous-edematous plates of diverse diameter covering 90% of the upper and lower extremities (Figure 1). The ailment had 2 years' evolution and a progressive course. The patient was diagnosed in private practice as having atopic dermatitis. After exacerbation of symptoms, he was treated with deflazacort and hydroxychloroquine with no improvement. Results from lesion biopsies revealed sarcoidal granulomas and the patient was therefore referred to the dermatology department at the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán for further study and treatment with the presumptive diagnosis of mycosis fungoides vs sarcoidosis.