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.

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

De-novo Ulceration - Rare Lazarine Leprosy - like Presentation of Borderline Lepromatous Hansen's Disease

Leprosy is caused by Mycobacterium leprae, which has a distinct predilection for cooler parts of the body. There are many forms of leprosy that range from the mildest indeterminate form to the most severe lepromatous type. The type of disease an individual develops depends on the host immune status; with tuberculoid type being seen in those with good immunity and lepromatous form in individuals with poor immunity to leprosy bacillus. Deep ulcers may occur in association with erythema nodosum leprosum, Lazarine leprosy and Lucio phenomenon. We report a male who presented with multiple acute onset foul smelling stellate ulcers over bilateral extremities. He was diagnosed as Borderline lepromatous leprosy (not in reaction) on the basis of histopathology. This type of presentation has not been reported till date.

Leprosy in Pregnancy: A Case Series in 4 Dermatology Clinics in Malaysia

IntroductionManagement of leprosy in pregnancy is challenging. Here we aim to describe the clinical characteristicsand the management of leprosy in pregnancy.MethodsThis is a retrospective study on pregnant women with leprosy managed in the Department ofDermatology in Hospital Kuala Lumpur, Hospital Pulau Pinang, Hospital Sultanah Bahiyah andHospital Queen Elizabeth between 1994 and 2015.ResultsThere were ten patients with 12 pregnancies with a median age of 27.5 years (range: 16-33). Fivewere foreigners. There were four cases of lepromatous leprosy, two cases of borderline lepromatous,two cases borderline tuberculoid leprosy and one case each for tuberculoid and mid borderline leprosy.Seven pregnancies (58%) were documented to have reactions. Four reversal reactions (33%), threeerythema nodusom leprosum (25%) and two Lucio’s phenomenon (17%) were documented. Bothpatients with Lucio’s phenomenon had undiagnosed leprosy and presented with preterm labour,anemia, oligohydramnios and intrauterine growth restriction. Mortality was recorded in one patientdue to dapsone induced hypersensitivity syndrome complicated with septicaemia. All patients wereprescribed multidrug therapy but in three pregnancies, the patients chose to defer the treatment. Therewas a spontaneous miscarriage at second trimester and a case of early neonatal death. The neonatalcomplications recorded for the 10 live deliveries were low birth weight, jaundice and clofazimineinducedhyperpigmentation.ConclusionThe majority of our patients with leprosy had complications throughout the pregnancies. Earlydetection and prompt treatment can prevent unfavorable fetal outcome & threatened maternal health.

Recurrent ulcerations and neuropathy in a Filipino child with previously undiagnosed leprosy: A case of Lucio phenomenon in the Philippines

@#<p style="text-align: justify;">Lucio phenomenon is a distinct necrotizing lepra reaction arising from diffuse lepromatous leprosy that may easily be mistaken for other systemic conditions. We report a 17-year old female admitted for a 4-year history of recurrent, extensive ulcerations with crusting and purulent discharge, involving the arms, legs, face, and back. Clinical presentation, slit skin smear and skin biopsy confirmed the diagnosis of Lucio phenomenon and treatment with multidrug therapy resulted in resolution of the ulcerations.</p>

Lepromatous leprosy and human immunodeficiency virus co-infection associated with phenomenon of Lucio versus immune reconstitution inflammatory syndrome / Lepra lepromatosa y coinfección con el virus de la inmunodeficiencia humana asociada a fenómeno de Lucio versus síndrome inflamatorio de reconstitución inmune

Diffuse lepromatous leprosy (DLL) is a severe clinical outcome of lepromatous leprosy (LL). The aetiologic cause is believed to be different from Mycobacterium leprae. A new species, Mycobacterium lepromatosis, was identified from a group of Mexican patients with DLL, and severe leprosy reactional state type 3 (Lucio's phenomenon). However, a total sequencing of its genome is necessary to prove the existence of this new species. This is a report on a non-typical Colombian case of leprosy - HIV coinfection, associated with an immune reconstitution inflammatory syndrome clinically compatible with a leprosy reaction type 3 or Lucio's phenomenon.

La lepra difusa (LLD) es una variedad de la lepra lepromatosa (LL), frecuente enMéxico. El agente etiológico se cree que es diferente a Mycobacterium leprae y se considerauna especie nueva denominada Mycobacterium lepromatosis, hecho que no se ha comprobado.El reporte de este caso se realiza para dar a conocer el cuadro clínico atípico que presentóuna paciente colombiana con coinfección VIH---LL variedad difusa (LLD), asociado a síndromede reconstitución inmunológica, compatible clínicamente con una leprorreacción tipo 3 o fenó-meno de Lucio.

Lepra reactions: A 10-year retrospective analysis

Introduction Leprosy is a chronic granulomatous infection caused by Mycobacterium leprae. Drug treatment is effective in eradicating the bacilli but does not prevent lepra reaction. Despite much attention being focused on the problem of lepra reactions, very limited data has been published on the epidemiology of lepra reactions especially this part of the world. The aim of the study is to improve the understanding of lepra reaction and to determine the demographics and clinical patterns of lepra reactions in Penang General Hospital. Materials and Methods This retrospective study covers a 10-year period from 1997 to 2006. Demographic characteristic and clinical patterns of lepra reactions were analysed with SPSS 13.0 version. Results Of the 95 patients who were enrolled in the study, 67 (70.5%) were male and 28 (29.5%) were females. The mean age at presentation was 40.4 ± 17.9 years (range 3-91 years). There were 35 Malays (36.8%), 34 Chinese (35.8%), 5 Indians (5.2%) and 21 foreigners (22.2%). 35.8% of patients presented with LL (n=34), 18.9% BT (n=18), 17.9% TT (n=17), 13.7% BB (n=13) and 13.7% BL (n=13). In our series, the lepra reaction rate among leprosy patient was 51.6% (n=49). Among those with lepra reaction, 53.1% cases were type 1 reaction (n=26), 44.9% cases were type 2 reaction (n=22) and 2.0% cases were Lucio phenomenon (n=1). Common manifestations observed in lepra reaction were worsening of skin lesions (100%), inflammatory oedema of hands, feet and face (53.1%), nerve pain (46.9%), fever (20.8%) and nerve tenderness (20.4%). Only 4 cases had involvement of other organs like the eye and joint. 30.6% of the reactions observed in our cohort were severe. Type 1 reaction commonly involved those in borderline spectrum whereas type 2 reaction commonly involved those in the lepromatous spectrum. Lepra reactions occurred before treatment (24.5%), during treatment (71.4%) or even after treatment has been stopped (4.1%). Most of the lepra reactions occurred during the treatment period especially the first 12 months of therapy. Conclusion Our study showed a more severe and higher reaction rate compared to other studies. Lepra reaction is a common presentation of leprosy. Type 1 reaction commonly involved those with borderline disease but type 2 reaction commonly involved those with lepromatous spectrum of disease. Lepra reaction occurred before, during and even after the treatment has stopped. Most of the lepra reactions occurred during treatment period especially the first 12 months of therapy.