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

Abstract

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.

New insights at the interface between leprosy and immune-mediated rheumatic diseases

“…LP is an acute, life-threatening, necrotizing skin reaction usually observed in untreated patients with diffuse lepromatous forms with high bacillary loads. In most reports, LP occurred as the first overt manifestation of leprosy in patients previously undiagnosed; in a minority of cases (<15%), LP occurred during the treatment or even after its completion ( 56 , 57 ). Clinically, LP is characterized by outbreaks of painful, sharply delineated erythematous-purpuric macules with central necrosis and subsequent irregular skin ulceration in limbs (mainly), trunk, and face, leaving stellar scars; the skin is diffusely infiltrated; and up to 50% of the patients have fever.…”

“…Considering the rarity of vasculitis and the difficulty of its differential diagnosis, a tissue biopsy should be performed in all cases, maintaining high clinical suspicion in endemic areas ( 58 ). The histopathology reveals a large number of acid-fast bacilli (AFB) in the endothelium, which is proliferated with areas of fibrinoid necrosis, vascular thrombotic occlusion with ischemic epidermic necrosis, discrete lympho-histiocytic inflammatory infiltrates, abundant AFB in macrophages that infiltrate the walls of skin vessels (along with AFB themselves), and leukocytoclastic vasculitis ( 56 , 57 ). Anemia, leukocytosis, and elevated acute phase reactants are common.…”

The leprosy reaction is associated with salivary anti-Porphyromonas gingivalis IgA antibodies

“…There is a type of reaction known as de Lucio’s phenomenon or type 3 reaction that is characterized by the development of severe, necrotizing, cutaneous lesions with a high mortality rate. This phenomenon is rare and occurs in patients with diffuse lepromatous leprosy (Frade et al 2022 ).…”

Lucio´s phenomenon. About a case

“…En el estudio histológico destacan las alteraciones en los vasos dérmicos con engrosamiento e infiltrado inflamatorio en las paredes y trombos de fibrina endoluminales. Al presentarse esta reacción en pacientes con Lepra Lepromatosa en la baciloscopía se observan generalmente abundantes bacilos (5). Este fenómeno puede ser difícil de reconocer, especialmente en países no endémicos, lo que puede llevar a un retraso en su diagnóstico y manejo (6,7).…”

“…Este fenómeno puede ser difícil de reconocer, especialmente en países no endémicos, lo que puede llevar a un retraso en su diagnóstico y manejo (6,7). La mayoría de los casos reportados provienen de Brasil, India y Argentina (5). En Paraguay, aproximadamente el 2% de los casos de Lepra diagnosticados presentan esta reacción, siendo observada sobre todo en el sexo masculino y, en la mayoría de los casos, como debut de la enfermedad (8).…”