Lucio Phenomenon: An Unusual Case of Skin Necrosis.

Introduction: Lucio leprosy is a non-nodular diffuse type of lepromatous leprosy first described by Lucio and Alvarado. Lucio phenomenon is a rare vasculonecrotic reaction characterized by cutaneous necrosis with minimal constitutional features. Case Presentation: We describe an unusual case of a 53-year-old man from Central India who had blisters, ulcers, and widespread erosions on his foot, forearms, and arms. The diagnosis of lepromatous leprosy with the Lucio phenomenon was established after thorough evaluation by clinical findings, histopathological findings, and slit-skin smear examination. Conclusion: Lucio phenomenon is an uncommon cause of cutaneous infarction and necrosis. Primary care physicians should keep a high index of suspicion in patients with cutaneous necrosis and minimal constitution features. Since leprosy is a relatively curable disease, primary care physicians should think of a rare form of lepromatous leprosy presenting with cutaneous necrosis, especially in non-endemic zones.

Presentation of Hansen's disease as Lucio phenomenon in an Indian male.

Lucio phenomenon is a reactional state described in patients with Lucio leprosy and in a few cases of lepromatous leprosy; it is rarely seen outside Mexico and Central America. We report a case of 35-year old labourer who presented with clinical features classical of Lucio phenomenon without any pre-existing cutaneous nodules or infiltrative lesions of either Lucio or lepromatous leprosy. This case report demonstrates the need to consider Lucio phenomenon in patients presenting with clinical features of medium vessel vasculitis even in areas not endemic for Lucio leprosy.

Leprosy Caused by Mycobacterium lepromatosis.

OBJECTIVES: Leprosy is caused by Mycobacterium leprae or Mycobacterium lepromatosis. This study reviews literature on M lepromatosis and reports on a Mexican family with this infection. METHODS: The review included all primary studies. Family history and surveys were used to uncover the infection cluster. Genome-based differential polymerase chain reactions were designed to detect etiologic agents. RESULTS: Since the discovery of M lepromatosis in 2008, 154 cases of M lepromatosis infection from 11 countries in the Americas and Asia have been reported, with most cases coming from Mexico. These cases included diffuse lepromatous leprosy (DLL) and other leprosy forms. Genomes of M lepromatosis strains have lately been sequenced, revealing 3,271,694 nucleotides and approximately 15% mismatches with M leprae. The Mexican family with leprosy involved the grandfather, mother, and 2 grandsons. The index was the oldest grandson, who manifested DLL and likely contracted the infection from his maternal grandfather approximately 13 years earlier. Family surveys diagnosed DLL in the index patient's mother and borderline leprosy in his brother; both were likely infected by the index patient. M lepromatosis was identified from archived biopsies from the index patient and his mother, while M leprae was excluded. CONCLUSIONS: M lepromatosis is a significant cause of leprosy in Mexico and requires better surveillance and control.

Fenómeno de Lucio como forma de presentación de enfermedad de Hansen / Lucio's phenomenon as a presentation of Hansen disease / Fenômeno de Lucio como forma de apresentação da hanseníase

Resumen: Introducción: la enfermedad de Hansen es una enfermedad infecciosa crónica, causada por Mycobacterium leprae, que afecta principalmente piel y nervios periféricos. Las reacciones leprosas son eventos agudos que se asocian a un aumento de la morbimortalidad de la enfermedad. Objetivo: presentar el caso clínico de un paciente con fenómeno de Lucio, a través del cual se llegó al diagnóstico de enfermedad de Hansen, y remarcar la importancia de tener presente esta enfermedad, poco frecuente en nuestro país, para su correcto diagnóstico. Discusión: el fenómeno de Lucio es un tipo de reacción leprosa mediada por inmunocomplejos. Se caracteriza clínicamente por máculas o placas eritematovioláceas, de aparición súbita, que evolucionan a úlceras necróticas y curan dejando cicatrices estrelladas atróficas. De no mediar tratamiento, puede ser fatal, debido a sobreinfección y sepsis. Este se basa en el tratamiento específico de la infección, asociado a prednisona y un correcto manejo de las heridas.

Summary: Introduction: Hansen disease is a chronic infectious disease caused by Mycobacterium leprae, which mainly affects the skin and peripheral nerves. Leprosy reactions are acute events associated to an increase in the morbimortality of the disease. Objective: the study aims to present the clinical case of patient with Lucio´s phenomenon, which allowed the diagnosis of Hansen disease, and to emphasize on the importance of having this disease in mind for an appropriate diagnosis, despite it being rather unusual in our country. Discussion: Lucio´s phenomenon is a kind of leprosy reaction mediated by immune complexes. Clinically, it is characterized by the sudden onset of macules or blue hemorrhagic plaques, with a rapid evolution to necrotic ulcers, and it heals leaving star-shaped atrophic scars. If it is not treated, it may be fatal due to superinfection and sepsis. Treatment is based on specific medication for the infection, associated to prednisone and the correct handling of injuries.

Resumo: Introdução: a hanseníase é uma doença infecciosa crônica causada pelo Mycobacterium leprae, que acomete principalmente pele e nervos periféricos. As reações hansênicas são eventos agudos que estão associados ao aumento da morbimortalidade da doença. Objetivo: apresentar o caso de um paciente com fenômeno de Lúcio, por meio do qual se chegou ao diagnóstico de hanseníase, e ressaltar a importância de se ter em mente esta doença, rara em nosso meio, para seu correto diagnóstico. Discussão: o fenômeno de Lúcio é um tipo de reação hansênica mediada por imunocomplexos. Caracteriza-se clinicamente por máculas ou placas eritêmato-violáceas de início súbito que evoluem para úlceras necróticas e cicatrizam, deixando cicatrizes estreladas atróficas. Sem tratamento pode ser fatal, devido a superinfecção e sepse; a terapia está baseada no tratamento específico da infecção, associado à prednisona e no manejo correto da ferida.

Necrotizing Soft Tissue Infection of the Upper Extremity as a Manifestation of Hansen's Disease.

Hansen's disease is a well-described, largely historic infection that is caused by Mycobacterium leprae. Lucio's phenomenon is an aggressive, rare form of untreated lepromatous leprosy characterized by diffuse cutaneous lesions and systemic symptoms. To date, cases of necrotizing soft tissue infection in the setting of leprosy have rarely been reported in the literature. We present the case of a 51-year-old man with no known past medical history who presented for the evaluation of acute-on-chronic left upper extremity ulceration, soft tissue swelling, and pain. The patient was diagnosed with necrotizing soft tissue infection of the left upper extremity and underlying multibacillary lepromatous leprosy with Lucio's phenomenon. He underwent dermatofasciectomy of the affected extremity, followed by staged soft tissue coverage, including dermal allograft placement. Proper antibiotic management was also undertaken. In this article, we describe a case of previously undiagnosed leprosy with Lucio's phenomenon manifesting as necrotizing fasciitis of the upper extremity.

Lucio's phenomenon: A systematic literature review of definition, clinical features, histopathogenesis and management.

Leprosy is a chronic disease with clinical presentations according to the immunologic spectrum. Lepromatous form is the most advanced, with the highest transmissibility and risk of causing disabilities. Lucio's phenomenon is a rare manifestation among lepromatous patients with a rapid and severe evolution and high mortality. It is difficult to differentiate from ulcerative/necrotic erythema nodosum leprosum and has no consensus on how it should be treated. This article is a qualitative review of the literature after the introduction of multidrug therapy, aiming to bring consensus related to the clinical, laboratory and histopathological diagnostic criteria of the disease and its management.

Lucio Phenomenon: Sequelae of Neglected Leprosy.

Lucio phenomenon is a potentially fatal leprosy reaction seen in pure, primitive, and diffuse form of lepromatous leprosy. It is a globally restricted phenomenon that occurs in Mexico and Costa Rica; however, sporadic cases have been reported elsewhere. We report a rare case of a 50-year-old Indonesian, who resided in Malaysia for the past 33 years, which presented with extensive ulcers on all four limbs for 3 weeks, as well as a 16-year history of skin changes and alopecia. Full evaluation established the diagnosis of lepromatous leprosy with Lucio phenomenon. Delay in diagnosis leads to significant disabilities and disease transmission within the community. Therefore, primary care practitioners, even in non-endemic countries, should be wary of this atypical feature of leprosy to prevent complications since it is a highly curable disease.

Lucio's leprosy and Lucio's phenomenom, histoid leprosy, nodular leprosy of childhood, primary neural leprosy, and diagnosis using fine needle aspiration cytology

In this chapter, some special forms of the clinical and histopathological presentation of leprosy are discussed: Lucio's leprosy and Lucio's phenomenon, histoid leprosy, nodular leprosy or childhood, and primary neural leprosy. The main clinical and histopathological characteristics of these forms and the condition under which they appear within the entire spectrum of leprosy and its reaction phenomena are presented. in addition, the main differential clinical and pathological diagnoses of each of these lesions are discussed. The use of fine-needle aspiration cytology for the diagnosis os leprosy, including its reaction phenomena, has also been addressed. The identication of the histopathological features of these special forms of leprosy is important to confirm the clinical diagnosis for guiding treatment and preventing the possible misinterpretation of clinical and histopathological findings.

Mycobacterium leprae: Pathogenesis, diagnosis, and treatment options.

Mycobacterium leprae is known to cause leprosy, a neurological and dermatological disease. In the past 20 years, 16 million leprosy cases have been recorded and more than 200,000 new cases were registered each year, indicating that the disease is still progressing without hindrance. M. leprae, an intracellular bacterium, infects the Schwann cells of the peripheral nervous system. Several types of leprosy have been described, including indeterminate, tuberculoid, borderline tuberculoid, mid-borderline, borderline lepromatous and lepromatous, and three different forms of leprosy reactions, namely type 1, 2 and 3, have been designated. Microscopic detection, serological diagnostic test, polymerase chain reaction and flow tests are employed in the diagnosis of leprosy. The recommended treatment for leprosy consists of rifampicin, dapsone, clofazimine, ofloxacin and minocycline and vaccines are also available. However, relapse may occur after treatment has been halted and hence patients must be educated on the signs of relapse to allow proper treatment and reduce severity. In this review, we depict the current understanding of M. leprae pathogenicity, clinical aspects and manifestations. Transmission of leprosy, diagnosis and treatment are also discussed.

Long-term ulcerations caused by Mycobacterium lepromatosis.

Patients with leprosy rarely present ulcerated lesions that can appear during reactional states like Lucio's phenomenon (LP), as in our case. LP is a rare complication of multibacillary leprosy due to massive bacilli invasion of endothelial cells causing a thrombotic syndrome. The initial macular lesion is purpuric followed by multiple infiltrated papules and nodules, some of them ulcerated, associated to loss of sensation on lower limbs. The importance of recognizing ulcers as a specific cutaneous manifestation of leprosy allows early diagnosis and treatment, and therefore avoiding the development of disabilities and persistence of illness. Infection by Mycobacterium lepromatosis is associated with LP and it should be especially sought in patients from endemic areas.

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.

Leprosy: Treatment and management of complications.

In the second article in this continuing medical education series, we review the treatment of leprosy, its immunologic reactions, and important concepts, including disease relapse and drug resistance. A fundamental understanding of the treatment options and management of neuropathic sequelae are essential to reduce disease burden and improve patients' quality of life.

Diffuse multibacillary leprosy patient with Lucio's phenomenon and positive anticardiolipin antibody misdiagnosed as lupus erythematosus panniculitis in the People's Republic of China.

Lucio's phenomenon (LP) is a special reactional state associated with diffuse multibacillary leprosy; both exhibit a limitative global distribution mainly in Mexico and Central America. We report a case of a 28-year-old female leprosy patient in the People's Republic of China, together with LP and positive anticardiolipin antibody, characterized by vascular thrombosis and invasion of blood vessel walls by leprosy bacilli, causing extensive skin ulcers and followed by a large number of atrophic scars.

Lucio phenomenon mimicking antiphospholipid syndrome: The occurrence of antiphospholipid antibodies in a leprosy patient.

Lucio phenomenon is an atypical reaction of leprosy, characterized by vasculitic lesions that can mimic antiphospholipid syndrome (APS) clinically. Distinguishing the two can be difficult as antiphospholipid autoantibodies may be present in patients with leprosy. We report on a 32-year-old female patient presenting with a sudden onset of fever, hemorrhagic bullae, and skin necrosis on her lower legs. She was treated for APS due to the presence of antiphospholipid antibodies but had an inadequate response. A skin biopsy revealed thrombotic vasculopathy and necrotizing vasculitis associated with aggregation of foam cells in the perivascular area and subcutis, with acid-fast bacilli in the histiocytes and blood vessel walls. Direct immunofluorescence showed IgM, C3, and fibrinogen deposition in the superficial and deep dermal blood vessels. The pathology confirmed the diagnosis of Lucio phenomenon, and appropriate therapy was given. It is essential to evaluate the patient comprehensively, including clinical, serological, and pathological aspects, to obtain the correct diagnosis.

Lucio Phenomenon: A Rare Presentation of Hansen's Disease.

Lucio phenomenon (LP) is a rare reactional state seen in cases of diffuse lepromatous leprosy. Lucio leprosy is a pure, primitive, and diffuse form of lepromatous leprosy. It is observed almost exclusively in Mexico and Central America and is considered a globally restricted phenomenon. However, isolated cases are being reported worldwide. Patients with Lucio leprosy often present with manifestations of LP, which includes purpuric macules with multiple and extensive areas of ulceration with bizarre-patterned, angulated borders mainly affecting the extremities. LP is difficult to recognize, especially in nonendemic countries, which can lead to a delay in its diagnosis and management. We report a case of LP due to its occurrence in the classical form of lepromatous leprosy and rarity in North India.

Fenómeno de Lucio: a propósito de un caso / Lucio's phenomenon: a case report

Se presenta caso de paciente masculino de 67 años de edad, fototipo III, con antecedentes patológicos personales de lepra lepromatosa en tratamiento, que acude a consulta especializada de Dermatología en el Hospital Guillermo Fernández Baquero (La Habana, Cuba) por lesiones ulcerosas diseminadas. Se realizan complementarios y se concluye caso como fenómeno de Lucio. Resaltando la infrecuencia de este eritema necrosante y la severidad de los estados reactivos de la lepra pues son procesos distintos pero destructores de los tejidos, supuestamente dirigidos por el sistema inmunológico que aumentan en gran medida la morbimortalidad de esta enfermedad

A case of a 67 year old male patient, phototype III, with a personal pathological history of lepromatous leprosy in treatment is presented at a specialized dermatology clinic at Guillermo Fernández Vaquero hospital for disseminated ulcerative lesions. Complementary are performed and case is concluded as a phenomenon of Lucio. Highlighting the infrequency of this necrotizingerythema and the severity of the reactive states of leprosy are different but destructive processes of the tissues, supposedlydirected by the immune system that greatly increase the morbidity and mortality of this disease.

Diffuse Multibacillary Leprosy of Lucio and Latapí with Lucio's Phenomenon, Peru.

Diffuse multibacillary leprosy of Lucio and Latapí is mainly reported in Mexico and Central America. We report a case in a 65-year-old man in Peru. He also had Lucio's phenomenon, characterized by vascular thrombosis and invasion of blood vessel walls by leprosy bacilli, causing extensive skin ulcers.

Reacciones leprosas / Leprosy reactions

Resumen Entre 30-50 % de pacientes con lepra pueden llegar a presentar reacciones inmunológicas, que son eventos agudos que interrumpen el curso crónico de la lepra y son importantes debido a que pueden causar a largo plazo secuelas graves, como el daño permanente de nervios, lo que conlleva a discapacidad y deformidad. Se revisan los diferentes aspectos clónicos y terapéuticos de las reacciones lepromatosas con el fin de profundizar en el conocimiento actual de estas complicaciones de difícil diagnóstico y que son prioritarias para prevenir discapacidades.

Abstract Between 30-50% of patients with leprosy could have immune reactions, which are acute events that disrupt the chronic course of leprosy and are important because they can cause serious long-term consequences such as permanent damage nerve, leading to deformity and disability. We review the different clinical and therapeutic aspects of lepromatous reactions in order to deepen the current knowledge of these complications of a difficult diagnosis and are the priority to prevent disabilities.