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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.

Background: Lucio's phenomenon (LP) is a rare variant of leprosy reaction, with the clinical manifestation of “necrotizing erythema.” LP was observed in patients with lepromatous leprosy who have not received or completed the treatment, and it is especially evident in patients with diffuse lepromatous leprosy (DLL), known as Lucio-Latapi’s leprosy. LP occurs due to M. leprae invading the blood vessels, causing endothelial damage that leads to thrombosis, ischemia, infarction, and tissue necrosis. The clinical features of the disease are erythematous lesions that can be accompanied by vesicles or bullae, with ulcers forming scar tissues. Histopathological examination can help establish the diagnosis of LP. Until now, there is still controversy about LP management. Multidrug therapy for multibacillary leprosy (MDT-MB) is the preferred line of treatment. Lucio-Latapi’s leprosy and LP are commonly found in Mexico and Central America, but rarely reported in Indonesia. Case Illustration: We report here the clinical description and development of six patients with LP observed in the tertiary referral hospital in Indonesia over a five-year period from 2013 to 2017. Discussion: All patients were diagnosed using clinical and histopathological examination, and all of them presented with ulceration and vasculitis. They were treated with MDT-MB WHO regimens and systemic corticosteroids. Five patients were alive, and one died due to extensive cutaneous lesions that lead to sepsis. Conclusion: Early diagnosis and prompt institution of multidrug therapy with systemic corticosteroids may improve the prognosis and outcome of LP. Keywords: Indonesia, Lucio-Latapi’s phenomenon, leprosy, reaction

Background: Lucio’s phenomenon (LP) is a severe necrotizing cutaneous reaction that occurs in patients with Lucio ’s leprosy. The exact pathomechanism is not fully understood, but typically abundant acid-fast bacilli in the walls of blood vessels point to direct perturbation of blood supply to the epidermis. We report a case of LP where epidermal necrosis occurred in the absence of vascular invasion by mycobacteria, raising the question whether this was an epiphenomenon or a true LP. Case Illustration: A 34-year-old male was referred for an episodic swelling of his hands and feet that persisted for two years. There were signs of leprosy (diffuse shiny skin lesions, anesthesia, and anhidrosis of the extremities) with purpuric patches on lower extremities. The slit-skin smear test revealed a high index of acid- fast bacilli. Histopathological examination revealed epidermal necrosis and leukocytoclastic vasculitis without demonstrable bacillus. WHO multidrug regimen for leprosy and corticosteroids successfully cured the patient and prevented ulcer development. Discussion: Despite the presence of classical LP characteristics clinically and histologically, mycobacterium was absent in the vessels’ walls . We hypothesized that, in LP, vascular impairment might also be secondary due to antigen – antibody reaction and hypercoagulable state. Conclusion: Purpuric patches in Lucio ’s leprosy might be the first sign of skin necrosis or vascular damage (purpura and ulceration). Besides the antimycobacterial drugs, anti-inflammatory drugs should be administered. Laboratory test for hemostasis might be advised. Keywords: Lucio’s phenomenon, mycobacteria, histopathology, vasculitis

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.

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, predominantly affecting the skin and peripheral nerves. Lucio phenomenon is a rare reactional state presenting in patient with lepromatous leprosy and described as acute cutaneous necrotising vasculitis. We discussed the case of a 38-year-old male patient presenting with oedematous and dusky discolouration of bilateral hands and feet associated with diffuse facial involvement. His skin condition gradually worsened to form multiple ulcers with bizarre shaped purpuric lesion over bilateral upper and lower limbs, trunk, and face. Histopathological examination of the skin biopsy showed multiple acid-fast bacilli and diagnosis of Lucio’s phenomenon was made in the background of lepromatous leprosy. He was treated with multi drug therapy (MDT) as recommended by the WHO guidelines. A better understanding of rarer manifestation of this illness is important for early diagnosis and to prevent significant morbidity associated with it.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S20

Leprosy is a complex disease which will affect in many aspects of the patient. In dr. Soetomo hospital, there were two cases of pregnancy with leprosy and Lucio’s phenomenon from 2014 until 2018. Both had been receiving multidrug therapy (MDT) before pregnancy but stopped due to lack of compliance. First case was resulted with term. Second case was admitted with worse condition than the first case, fetal growth restriction and ended with preterm delivery probably because the severity of the case. Lucio’s phenomenon incidence is increased in pregnancy due to immunodeficient condition. This serial case report shows that the initial diagnosis and optimum treatment of leprosy is very important especially in women of child-bearing age.

Lucio’s Phenomenon A 48-year-old man presented with fever and blistering skin lesions. Multiple acid-fast bacilli were detected on biopsy of the skin, and a diagnosis of Lucio’s phenomenon, a react...

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.

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.

Lucio’s phenomenon is an uncommon reaction characterized by severe necrotizing cutaneous lesions that occurs in patients with Lucio’s leprosy and lepromatous leprosy. It is considered by some authors as a variant of type 2 or 3 reaction. Death can occur because of blood dyscrasia or sepsis. Precipitating factors include infections, drugs and pregnancy. We report a 31-year-old female patient exhibiting both clinical and histopathological features of lepromatous leprosy and Lucio’s phenomenon presenting favorable response to treatment. We complement our report with a literature review of the Brazilian cases of Lucio’s phenomenon published in Portuguese and English.

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.

Lucio's phenomenon (LPh) is considered a necrotizing panvasculitis and a variant of leprosy Type 2 reaction, clinically characterised by necrotic-haemorrhagic lesions on the extremities and trunk. LPh is observed in diffuse lepromatous leprosy (DLL or Lucio-Latapi leprosy). This is a distinct form of lepromatous leprosy (LL) reported mainly in Mexico. Anti-phospholipid antibody syndrome (APS) has been rarely described in LPh. We report a case of Lucio-Latapi leprosy with LPh observed in a patient from the province of El Oro in Ecuador, who presented clinical manifestations of long standing DLL (non-nodular infiltration of the skin, collapse of the nasal pyramid, madarosis, atrophy of the earlobes), of LPh (necrotic-haemorrhagic macules with irregular shapes) and of APS (necrosis of the right big and second toe). Histopathology showed perineural and periadnexal foamy macrophages with numerous bacilli (diagnostic of LL) in the subcutis, a mild lobular panniculitis with a large subcutaneous vessel infiltrated by macrophages in the wall (typical of LPh) and vessels of the superficial and mid dermis occluded by thrombi but without signs of vasculitis (typical of occlusive vasculopathy as in APS). Our observations suggest that some cases of LPh may be associated with APS. Anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LA) should be tested in patients with LPh because this may have therapeutic implications.

Introduction: Leprosy is a chronic, multisystemic granulomatous infection caused by Mycobacterium leprae. Lucio’s phenomenon is an uncommon reaction consisting of a severe chronic necrotizing vasculitis. The purpose of this article is to present two cases of Lucio’s phenomenon and a review of the literature on its clinical presentation and management. Case Presentation: Two middle-aged men presented with ulcers and ecchymosis in lower extremities, with simultaneous peripheral nerve damage and leonine facies. Both were treated according to World Health Organization recommendations, with a favorable clinical response. Discussion: The clinical characteristics presented in this article make part of the classical description. A proper history and physical examination allowed for a correct diagnostic approach and prompt confirmation of diagnosis, despite the unspecific nature of these signs and symptoms. Conclusions: Leprosy and Lucio’s phenomenon are infrequent conditions that are difficult to diagnose. We suspect this condition to be under-registered. Awareness and a high clinical suspicion are necessary in endemic regions.

Lucio's phenomenon represents a serious cutaneous necrotizing reaction, which can occur with Lucio's leprosy and also in other forms of lepromatous leprosy. The authors discuss the case of a 63-year-old male patient presenting with ulcers and necrotic cutaneous lesions on the limbs, torso and face, associated with recurring sinusitis, hoarseness and a weight loss of 25 kilos over the last year. Bacilloscopy of intradermal scrapings showed a bacilloscopic index of 5 and the histopathological findings were compatible with the diagnosis of Lucio's phenomenon. The chosen treatment was exclusively multibacillary multidrug therapy, resulting in important dermatological improvement after one month of therapy. The authors present an impressive case of Lucio's phenomenon with an exceptional response to treatment exclusively with multibacillary multidrug therapy.

We report a 62-year-old farmer who consulted with multiple chronic wounds over his limbs and chest. Physical examination revealed multiple ulceration, madarosis and infiltration of ear lobes. Slit skin smear showed numerous acid-fast bacilli consistent with a diagnosis of lepromatous leprosy associated with Lucio’s phenomenon. Administration of thalidomide together with MDT resulted in a huge improvement.

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.

Leprosy is caused by the well-known Mycobacterium leprae and the newly discovered M lepromatosis . Here, the authors describe 2 cases of leprosy with unusual clinical presentation caused by M lepromatosis . The patients, a 32-year-old man and a 50-year-old woman, both of Mexican origin, manifested high fever, lymphadenopathy and florid skin lesions in the form of erythema nodosum and Lucio's phenomenon as the first clinical presentation. Heavy infiltration of acid-fast bacilli was identified in the tissues that led to the diagnosis of lepromatous leprosy or diffuse leprosy. The patients were treated with multidrug regimen and responded appropriately. From the lymph node tissue, the authors showed the bacillus to be M lepromatosis , not M leprae as presumed previously, by differential polymerase chain reactions and analysis of gene sequences. These cases add to the growing studies on this organism, expand its endemic regions in Mexico and provide more clinical insight.

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. Resumen: 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.

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.

Background Lucio’s phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio’s phenomenon in a previously undiagnosed leprosy patient who presented to us with its classical skin manifestations. Case presentation A 64 year old South Asian (Sri Lankan) male with a history of chronic obstructive airway disease presented to us with fever and cough. He had a generalized smooth and shiny skin with ulcerating skin lesions afflicting the digits of the fingers. The lesions progressed to involve the extremities of the body and healed with crusting. Based on the clinical and investigational findings Tuberculosis and common vasculitic conditions were suspected and excluded. The unusual skin manifestations prompted a biopsy, and wade fite stained revealed Mycobacterium bacilli. In context of the clinical picture and histological findings, Lucio’s phenomenon was suspected. A clinical diagnosis of Lucio’s phenomenon occurring in the backdrop of lepromatous leprosy was made. Conclusion Though leprosy is still a prevalent disease, it has manifestations that are not easily recognized or fully appreciated. Regional patterns of atypical manifestations should not limit better understanding of rarer manifestations as it will aid in clinching an early diagnosis and instituting prompt treatment, thereby reducing morbidity and mortality.

Lucio's phenomenon/erythema necroticans is a peculiar reaction pattern that occurs in untreated pure primitive diffuse lepromatous leprosy (PPDL) and/or relapsing leprosy recognized as spotted leprosy of Lucio. The small number of reported cases in the world literature suggests that it is fairly uncommon. Its clinical features are fairly characteristic and consist of extensive, bizarre, painful ulcerations of the skin, with constitutional symptoms being conspicuous by their absence. The clinical diagnosis is confirmed by microscopic pathology marked by proliferation and mobilization of polyblasts and histiocytes, dilatation, endothelial proliferation, luminal occlusion, and thrombosis of the superficial and mid-dermal blood vessels and demonstration of acid-fast bacilli in the blood vessel walls. Its precise pathogenesis is still unclear, but is believed to occur either through the usual or the alternate pathway of complement activation in the natural history of erythema nodosum leprosum. The clinical and immunological features of reactions in leprosy, including erythema nodosum leprosum, are well known and have been critically evaluated elsewhere.