Leprosy with type 1 reaction in a patient from Ontario, Canada without recent travel misdiagnosed as vasculitic neuropathy: a case report.

BACKGROUND: Leprosy is rare within non-endemic countries such as Canada, where cases are almost exclusively imported from endemic regions, often presenting after an incubation period of as many as 20 years. Due to its rarity and prolonged incubation period, diagnosis is often delayed, which may result in neurologic impairment prior to the initiation of treatment. In this report we describe a case that is novel in its incubation period, which is the longest reported to-date and may have contributed to diagnostic delay. The case also uniquely demonstrates the challenges of distinguishing leprosy reactions from new rheumatologic manifestations in a patient with established autoimmune disease. CASE PRESENTATION: We describe an 84-year-old male patient with rheumatoid arthritis on methotrexate and hydroxychloroquine, with no travel history outside Canada for 56 years, who presented in 2019 with new-onset paresthesias and rash. His paresthesias persisted despite a short course of prednisone, and his rash recurred after initial improvement. He underwent skin biopsy in May 2021, which eventually led to the diagnosis of leprosy. He was diagnosed with type 1 reaction and was started on rifampin, dapsone, clofazimine and prednisone, with which his rash resolved but his neurologic impairment remained. CONCLUSION: This case report serves to highlight the potential for leprosy to present after markedly prolonged incubation periods. This is especially relevant in non-endemic countries that is home to an aging demographic of individuals who migrated decades ago from endemic countries. The importance of this concept is emphasized by the persistent neurologic impairment suffered by our case due to untreated type 1 reaction. We also demonstrate the necessity of skin biopsy in distinguishing this diagnosis from other autoimmune mimics in a patient with known autoimmune disease.

Follow-up assessment of patients with Pure Neural Leprosy in a reference center in Rio de Janeiro-Brazil.

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.

Lepra neural pura. Aspectos diagnósticos en un caso clínico / Pure neural leprosy. Diagnostic aspects of a clinical case

Introducción. La lepra es una enfermedad infecciosa causada por la bacteria Mycobacterium leprae. Presenta especial avidez por la piel y los troncos nerviosos, y, de hecho, ambos se afectan en la mayor parte de los infectados. Se trasmite por exposición con enfermos y en ocasiones por reactivación. Una posibilidad inhabitual es la lepra neural pura, caracterizada por neuropatía, pero sin lesiones en la piel. Se describe un paciente con lepra neural pura y se revisan los aspectos diagnósticos. Caso clínico. Varón de 40 años, inmigrante, diagnosticado y tratado de lepra 20 años antes. Acudió por parestesias y disestesias dolorosas en las manos y las piernas sin lesiones en la piel. Se demostró mononeuritis múltiple aguda con principal afectación de cubitales. La enfermedad, tipificada como tuberculoide paucibacilar, se trató y en pocas semanas la mejoría fue evidente. Conclusiones. En este caso de lepra neural pura por reactivación, el diagnóstico temprano permitió un rápido tratamiento. Es recomendable la evaluación de la neuropatía integrada con criterios clínicos, electrofisiológicos y ecográficos. De este modo se consigue una alta sensibilidad y especialmente una precocidad en el diagnóstico y la instauración del tratamiento, y por consecuencia una mejor recuperación funcional (AU)

Introduction. Leprosy is an infectious disease caused by the bacteria Mycobacterium leprae. It is particularly prone to affect the skin and the nerve trunks and, in fact, both are compromised in most infected patients. It is transmitted by exposure to those with the disease and sometimes by reactivation. One uncommon possibility is pure neural leprosy, which is characterised by neuropathy, but without skin lesions. We report the case of a patient with pure neural leprosy and review the diagnostic aspects. Case report. A 40-year-old male, an immigrant who was diagnosed and treated for leprosy 20 earlier. The patient visited due to painful paraesthesias and dysesthesias in the hands and legs without the presence of any skin lesions. Acute multiple mononeuritis with mainly ulnar involvement was observed. The disease, typified as paucibacillary/tuberculoid, was treated and in a few weeks there was a clear improvement. Conclusions. In this case of pure neural leprosy due to reactivation, early diagnosis allowed timely treatment to be established. Evaluation of neuropathy together with clinical, electrophysiological and ultrasound criteria is recommended. By so doing, a high degree of sensitivity is achieved as well as allowing early diagnosis and treatment, and therefore a better functional recovery (AU)

Análise dos resultados de neurolises em pacientes com hanseniase / Analysys of the results of neurólises in patients with leprosy

O objetivo deste estudo é avaliar a eficácia da neurólise cirúrgica no tratamento da neurite periférica em pacientes com hanseníase. Trata-se de um estudo retrospectivo de vinte e cinco pacientes hansenianos submetidos à neurólise com média de idade de 39,4 anos. A perda sensitiva foi quantificada pelo método dos monofilamentos de Siemmens-Weistein e a dor foi informada pelo paciente através de escala numérica analógica. As variáveis foram analisadas pré e pós operatoriamente. O Teste da Binomial, utilizado para a análise comparativa entre as avaliações sensitiva, mostrou variação não significativa estatisticamente (p > 0,05) nas medições pré e pós-operatórias, indicando ausência de progressão da parestesia. Houve melhora significativa da dor em todos os pacientes. Conclusão: a neurólise cirúrgica foi eficaz no alívio da dor e interrompeu a progressão da perda sensitiva na neuropatia periférica de hansenianos.

Hand and foot syndrome secondary to capecitabine.

A 55-year-old woman on treatment with capecitabine and paclitaxel for breast carcinoma presented with history of a tingling sensation in her hands and feet with a progressive burning sensation. She also noted discomfort, minimal pain and stiffness while holding objects. On examination, there was patchy hyperpigmentation of both the palms and soles, and the dorsa of hands and feet. This was accompanied by a thickening of the skin more over the knuckles and toes. In addition there was a moist desquamation around the toes and over the palmar creases and a bluish discoloration of the lunulae of both thumbnails. She was diagnosed with hand and foot syndrome and started on pyridoxine and emollients. The finding of keratoderma noted in our patient is not seen commonly in hand and foot syndrome.

Novel presentation of lepromatous leprosy in an erythema gyratum repens-like pattern.

OBJECTIVES: Leprosy can have diverse cutaneous and occasionally perplexing presentations. We report an unusual case of lepromatous leprosy (LL) with annular lesions resembling erythema gyratum repens. REPORT: A 55-year-old man presented with a symmetrical, hypopigmented, and erythematous rash of bizarre appearance over the lateral aspect of the upper arm, and anterior and posterior aspects of the trunk of two months' duration. He gave a history of self-resolving episodes of bilateral pedal edema, and numbness and pricking sensations in both the hands and feet, which had occurred intermittently over the previous six years. An ulcer measuring 2 cm in size was present over the adjacent surface of the right first and second toes. The bilateral ulnar and radial cutaneous nerves were symmetrically thickened. RESULTS: Slit-skin smears revealed numerous acid-fast bacilli. Skin biopsy from the trunk showed collections of histiocytes, lymphocytes, and plasma cells in the dermis and around the blood vessels. The patient was diagnosed with LL and started on multibacillary multi-drug therapy. CONCLUSIONS: Lepromatous leprosy can have varied clinical manifestations and is often a great imitator. However, the skin smear positivity, even in normal skin, symmetrical cutaneous and peripheral nerve involvement, and histopathology in the present patient were indicative of LL. This report highlights a rare presentation of leprosy. Clinicians should be aware of these rare manifestations as lepromatous cases still occur in certain regions.

Trigeminal trophic syndrome: a rare entity.

Trigeminal trophic syndrome is a rare condition resulting from self-manipulation of the skin after a peripheral or central injury to the trigeminal system. The syndrome consists of a classic triad of anaesthesia, paraesthesia, and a secondary persistent or recurrent facial ulceration. We describe a 60 year-old woman who developed this syndrome as a sequel to the gasserian ganglion block for trigeminal neuralgia. She had also developed melasma within 1 year. A remarkable benefit was achieved by proper patient education and topical antibiotics which led to the healing of all ulcerations within 4 weeks. In the case reported here, the diagnosis of the trigeminal trophic syndrome was made primarily as a result of the physician's experience with the syndrome previously.

[Persistent type 2 lepra reaction (erythema nodosum) and clofazimine-induced lethal enteropathy]. / Eritema nudoso leproso persistente y enteropatía letal por clofazimina.

INTRODUCTION: Clofazimine enterophathy is a serious complication of clofazimine when used at high doses for treatment of type 2 lepra or or erythema nodosum leprosum. Objective. A woman is presented who had a delayed diagnosis of leprosy, persistent type 2 lepra reaction and lethal clofazimine enteropathy. MATERIALS AND METHODS: A 31-year-old woman presented leprosy symptoms over a 16-year period without medical diagnosis of her disease. During this period, type 2 lepra episodes occurred, but were not accurately diagnosed. These episodes became more severe during her second pregnancy. The patient and her family were interviewed, and her clinical history reviewed. RESULTS: After twelve years of medical consults, lepromatous leprosy was diagnosed, based on perforation of her nasal septum, with a bacterial index of 5. Her husband and a 12-year-old daughter have leprosy symptoms. During multidrug therapy, she presented with repeated type 2 lepra reaction episodes for which she received daily clofazimine 400 mg doses. Two months after this treatment, severe and frequent episodes of intense abdominal pain began to occur. These persisted for more than a year and were managed with in-hospital administration of several classes of painkillers and antispasmodic medication, including morphine. She also presented with sporadic diarrhea, constipation, nausea, weight loss and mesenteric adenopathies. She died finally due to this intestinal condition. No autopsy was performed. CONCLUSIONS: The patient's clinical presentation suggested a clofazimine-induced lethal enteropathy, a complication not previously seen in Colombia. This connection was not recognized by the medical officers that treated the patient.

[Lepra: an uncommon cause of infectious neuropathy]. / Lepra: causa infrecuente de neuropatía potencialmente tratable.

INTRODUCTION: Lepra is an uncommon disease within our setting. However, it was considered the most frequent cause of polyneuropathy only 50 years ago. CASE REPORT: We present the case of a 37 year-old woman who consulted due to paresthesias in both hands and feet, livedo reticularis and complaints of frequent hand lesions. Examination of the skin detected nodular lesions and the neurophysiological study confirmed distal symmetric sensitive polyneuropathy with axonal predominance. The skin biopsy also showed histocytic infiltrate and mycobacterium lepra type intracytoplasmatic bacilli. This led to the diagnosis of Multibacillary lepromatous leprosy. Multiple treatment was begun, according to the World Health Organization recommendations, with good evolution. In spite of the attempts to eradicate lepra, its incidence continues to be elevated, especially in endemic areas, among which the south of Spain is included. It is generally associated to overcrowding and low social-economic level. CONCLUSIONS: This is the last endemic case in the Valencian Community. The possibility of lepra should be considered among the possible causes of sensitive polyneuropathy, above all in patients in endemic areas.

Lepra: causa infrecuente de neuropatía potencialmente tratable / Lepra: an uncommon cause of infectious neuropathy

Introducción. La lepra es una enfermedad poco frecuente en nuestro entorno; sin embargo, hace tan sólo 50 años era considerada la causa más frecuente de polineuropatía. Caso clínico. Presentamos el caso de una mujer de 37 años que consulta por parestesias en manos y pies, livedo reticularis y quejas de frecuentes ulceraciones en las manos. En la exploración cútanea se detectaron lesiones nodulares y el estudio neurofisiológico puso de manifiesto una polineuropatía sensitiva simétrica distal de predominio axonal. La biopsia cutánea mostró un infiltrado histocitario y bacilos intracitoplasmáticos de tipo Mycobacterium leprae, lo que condujo al diagnóstico de lepra lepromatosa multibacilar. Se instauró tratamiento con politerapia, según recomendaciones de la Organización Mundial de la Salud, con buena evolución. A pesar de los intentos de erradicación de la lepra, su incidencia continúa siendo elevada, especialmente en áreas endémicas, entre las que se incluye el sur de España y, generalmente, asociada a hacinamiento y bajo nivel socioeconómico. Conclusiones. Éste es el último caso autóctono comunicado en la Comunidad Valenciana. Entre las posibles causas de polineuropatía sensitiva debe tenerse en cuenta todavía la posibilidad de la lepra, sobre todo en pacientes procedentes de áreas endémicas (AU)

Introduction: Lepra is an uncommon disease within our setting. However, it was considered the most frequent cause of polyneuropathy only 50 years ago. Case Report: We present the case of a 37 year-old woman who consulted due to paresthesias in both hands and feet, livedo reticularis and complaints of frequent hand lesions. Examination of the skin detected nodular lesions and the neurophysiological study confirmed distal symmetric sensitive polyneuropathy with axonal predominance. The skin biopsy also showed histocytic infiltrate and mycobacterium lepra type intracytoplasmatic bacilli. This led to the diagnosis of Multibacillary lepromatous leprosy. Multiple treatment was begun, according to the World Health Organization recommendations, with good evolution. In spite of the attempts to eradicate lepra, its incidence continues to be elevated, especially in endemic areas, among which the south of Spain is included. It is generally associated to overcrowding and low social-economic level. Conclusions: This is the last endemic case in the Valencian Community. The possibility of lepra should be considered among the possible causes of sensitive polyneuropathy, above all in patients in endemic areas (AU)

Leprosy: recognizing red flags.

A 28-year-old man from the Philippines presented with multiple papules and plaques symmetrically distributed on the arms and legs. This was associated with worsening paraesthesia of the hands and feet. A right common peroneal nerve decompression had been performed 2 years earlier. He was diagnosed with multibacillary leprosy on skin biopsy and subsequently treated with oral rifampicin, clofazamine and dapsone.

[Leprosy and severe neuropathy in two native Spaniards]. / Lepra y neuropatía grave en dos nativos españoles.

INTRODUCTION: Leprosy is a widespread infectious disease in humans that is endemic to regions with poor sanitary conditions, especially in cases of overcrowding, malnutrition and bad hygiene. The disease is characterised by dermopathy, which is quite typical, but above all by neuropathy, which often becomes the most important element. In most cases, alterations to nerves are defined by sensory deficits that are predominantly distal and multiple neuritis in areas where nerve entrapment has taken place. CASE REPORTS: Two patients, both native Spaniards, presented largely overlapping clinical pictures, that is, a history of 'glove and stocking' type paresthesias and dysesthesias going back months or even years and functional impotence, which gave rise to a very pronounced gait disorder. In the two cases, the immunological situation was determined to be borderline lepromatous leprosy. The neurophysiological study revealed the presence of severe, diffuse sensory-motor axonal polyneuropathy that was predominantly distal, and several entrapped nerves. The dermatological illness was greatly improved by the treatment. The same was partially true, although to a satisfactory extent, of the neurological disease. CONCLUSION: We describe the cases of two Spaniards with borderline lepromatous leprosy with no past history of the disease, in whom neuropathy was the predominant symptom. We highlight the speed with which the neuropathies progressed, probably due to a change in 'polarity', and the severity of the neurological deficits in comparison with the dermopathy, in an unusual immunological situation. The growing number of native patients in the first world, even when there is no relevant history, suggests that we should not think of leprosy as something only occurring in immigrant patients from places where it is endemic, although the epidemiological relationship has still not been determined.

Primary neuritic leprosy: a reappraisal at a tertiary care hospital.

AIM: To study the clinico-epidemiological profile of primary neuritic leprosy. METHODS: Retrospective analysis of the leprosy records of clinically diagnosed patients of PNL who attended the Leprosy Clinic from 2000 to 2004 was carried out for details of presenting complaints, age and sex distribution, duration, number of nerves involved and pattern of nerve enlargement, BI (skin), nerve abscess, and deformities. RESULTS: There were 32 (4.6%) patients of PNL out of a total of 686. Majority (56.2%) had complaints for less than a year. There were 29 (90.6%) males, and 3 (9.4%) females, with 15-30 years as the commonest age-group (65.6%) involved. Paraesthesia and numbness were the presenting complaints in 20 (62.5%), and motor deficit (paresis) in 11 (34.4%); deformities (claw hand, foot drop, trophic changes) were seen in 16 (50%) cases. Polyneuritic pattern was noted in 21 (63.56%) patients and mononeuritic in 11 (16.5%) with ulnar nerve as the most commonly enlarged nerve (63.6%). Nerve abscess was noted in 4 (12.5%) cases. Slit-skin smear was positive in 2 (6.2%) cases only. DISCUSSION: PNL continues to be common in India. Sensory complaints are early and more common. The disease is more common in males. Polyneuritic pattern was predominant, and the ulnar nerve was the most commonly involved nerve. Majority of the cases belong to the tuberculoid spectrum. Early diagnosis depends on complete neurological examination in order to reduce the sequelae of the disease.

Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology.

A 'syringomyelia-like' syndrome has been infrequently reported in neurological disorders such as Tangiers disease and lepromatous leprosy. This study reports a novel 'syringomyelia-like' syndrome in four adult male patients, which we have termed facial onset sensory and motor neuronopathy, or FOSMN syndrome, that appears to have a neurodegenerative aetiology. Clinical, neurophysiological and pathological data of four patients were reviewed, including the autopsy in one patient. Four male patients (mean age at onset 43), initially developed paraesthesiae and numbness in a trigeminal nerve distribution, which slowly progressed to involve the scalp, neck, upper trunk and upper limbs in sequential order. Motor manifestations, including cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy developed later in the course of the illness. Neurophysiological findings revealed a generalized sensory motor neuronopathy of caudally decreasing severity in all four patients. Autopsy in one patient disclosed loss of motoneurons in the hypoglossal nucleus and cervical anterior horns, along with loss of sensory neurons in the main trigeminal sensory nucleus and dorsal root ganglia. FOSMN syndrome appears to be a slowly progressive neurodegenerative disorder, whose pathogenesis remains to be determined.

[Neuropathy of leprosy: characteristics of cases in 1962-1995]. / Neuropatía leprosa: caracterización de la casuística entre 1962 y 1995.

INTRODUCTION: Neuropathy due to leprosy is the most frequent cause of peripheral nervous system disorders due to an infective agent, one of the commonest aetiologies of peripheral neuropathy, and also one of the few peripheral neuropathies which is curable. Hansen's disease, initially and predominantly involves the skin, so it is not usually seen by a neurologist; a neurologist sees cases which are difficult to diagnose and often after the condition has been ruled out by other doctors from other medical specialties. PATIENTS AND METHODS: In the National Institute of Neurology and Neurosurgery of La Habana, Cuba, 18 nerve biopsies were studied (during its history), that had been diagnosed as leprous neuropathy. We reviewed the clinical histories of all the patients admitted to this centre. The sample was characterized by: age, sex, neurological clinical picture, nerves most affected, skin lesions, electrophysiological studies and a description of the anatomopathological findings. RESULTS AND DISCUSSION: Most patients were male and all (100%) were adults of 'working age' (19-65 years). The most frequent neuropathic pattern was multiple mono-neuropathy (78%) and the quality, purely sensitive or mixed was predominantly sensitive (100%). The nerves most affected were the ulnar (10 patients) and median (6 patients). The most frequent skin lesions were anesthetic maculae. Anatomo-pathological study showed inflammatory infiltration in all patients and the bacillus was absent in only one case.