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.

Lepra dimorfa lepromatosa conyugal / Lepromatous dimorphic leprosy in spouses

Leprosy is a contagious, chronic infectious disease caused by Mycobacterium leprae. The immune response of the host to this bacillus is variable, determining different clinical forms of the same disease. Between the Lepromatous and Tuberculoid spectra, both stable clinical forms, the Dimorfo type can be presented, with great immunological instability, determining clinical characteristics, according to the pole to which most approaches. Leproatous dimorphic leprosy is characterized by brwn and violet macules, large number of lesions and less definition at its edges, variable sizes and alteration of sensitivity. Conjugal leprosy occurs in very few cases, knowing that intimate contaqct for a long time is an important factor, but has also demonstrated the fundamental role of immunity and genetics to acquire and develop the disease. We present two cases of lepromatous dimorphic leprosy in spouses, with 20 years of cohabitation, in which the same clinical presentation was found. Ths is an infrequent fact, given the low infectivity of the pathogen and the multiple varieties that could occur.

Asociación de Mycobacterium leprae viable con leprorreacciones / No disponible

La hipótesis de trabajo es que el Mycobacterium leprae (M. leprae) viable desempeña un papel crucial en la precipitación de la reacción tipo 1 (T1R) en lepra. Materiales y métodos: Se estudiaron un total de 165 nuevos pacientes multibacilares. Para demostrar la presencia de M. leprae se emplearon tres técnicas en las lesiones en reacción T1R (+), como son el crecimiento en la almohadilla plantar (MFP), detección inmunohistoquímica de la proteína secretora M. leprae Ag85, y 16s rRNA - empleando RT-PCR in situ. Como controles, se emplearon biopsias de BEC y lesiones sin reacción T1R (-). Hallazgos: Un número significativamente mayor de homogeneizados de biopsia de lesión obtenidos al inicio, de casos T1R (+) han presentado crecimiento en MFP, demostrando la presencia de bacterias viables comparado con T1R (-) (P = 0·005). En contraste, pocas biopsias BEC resultaron positivas tanto en T1R (+) como en T1R (-). Con respecto a Ag85, mientras que la positividad total fue más elevada en T1R (+) (74%), la positividad de la tinción (Grado ≥ 2+) era significativamente mayor en el grupo T1R (+) BT-BB 11/20 (55%). En las muestras de biopsias en casos de T1R recurrentes, tanto el Ag 85 como el 16s rRNA, la positividad (62% y 100%) fue mayor en el grupo T1R (+). Se propone que las bacterias viables son componentes esenciales en las T1R y la diferencia en la calidad de los bacilos y no la cantidad o la ratio de inactivos/viables desempeña un papel en la precipitación de T1R. En conclusión, los hallazgos demuestran que los bacilos «metabólicamente activos» de M. leprae son un componente/requisito fundamental de la T1R y la proteína secretora Ag 85, quizás sea el precipitador de T1R (AU)

The working hypothesis is that, viable Mycobacterium leprae (M. leprae) play a crucial role in the precipitation of Type 1 reaction (T1R) in leprosy. Material and Methods: A total of 165 new multibacillary patients were studied. To demonstrate presence of viable M. leprae in reactional lesion (T1R+), three tests were used concurrently viz. growth in the mouse foot pad (MFP), immunohistochemical detection of M. leprae secretory protein Ag85, and 16s rRNA - using in situ RT- PCR. Mirror biopsies and non reactional lesions served as controls T1R (-). Findings: A significantly higher proportion of lesion biopsy homogenates obtained at onset, from T1R (+) cases have shown unequivocal growth in MFP, proving the presence of viable bacteria, as compared to T1R (-) (P , 0·005). In contrast, few Mirror biopsies were positive in both T1R (+) and T1R (-). With respect to Ag85, while the overall positivity was higher in T1R (+) (74%), however the intensity of staining (Grade ≥ 2+) was disproportionately higher in T1R (+) BT-BB lesions 11/20 (55%). In the rebiopsies obtained during a repeat episode of T1R, Ag 85 as well as 16s rRNA, positivity (62% & 100%) was higher in T1R (+). It is inferred therefore «viable» bacteria are an essential component in T1R and difference in the quality of bacilli, not the quantity or the ratio of dead to viable play a role in the precipitation of T1R. In conclusion, the findings show that «metabolically active» M. leprae is a component/prerequisite and the secretory protein Ag 85, might be the trigger for precipitation of T1R (AU)

Hansen lepromatoso reaccional en un paciente pediátrico / No disponible

La lepra entre los niños es más frecuente de lo que se solía pensarse, sobre todo en lugares tropicales y subtropicales, como nuestro país. El hecho de que los síntomas iniciales de la lepra son muchas veces mal diagnosticados por pacientes y médicos, hace que el diagnóstico se establezca tardíamente, motivo por el cual el pediatra y dermatólogo deberían pensar en esta enfermedad para realizar de esta manera un diagnóstico precoz con el tratamiento específico. Se presenta el caso clínico de un niño de 13 años de edad con una forma multibacilar de lepra considerada poco frecuente en niños (AU)

Childhood leprosy is very frequent specially in tropical and subtropical areas, like our country. The fact is that initial symtoms are underestimated in routine examination and the diagnosis can pass unnoticed. The pediatricians and dermatologists should consider this disease to make an early diagnosis and to give the specific treatment . We present the case of a 13 year old child with multibacillar form of hansen’s disease, considered not usual in children (AU)

Reaction in leprosy: acute phase reactant response during and after remission.

Sera from 25 patients with type 1 (Lepra), upgrading and downgrading, and type 2 (erythema nodosum leprosum [ENL]) reactions were assayed, during the reaction and after its clinical remission, for changes in levels of alpha-1-antitrypsin (A1A) and C-reactive protein (CRP). The results were compared with those from normal healthy adults and patients of leprosy without history and/or clinical evidence of reaction. The A1A levels correlated better with changes in status of type 1 reaction; whereas CRP levels correlated well with alterations in type 2 reactions and were definitely superior to A1A in this situation for monitoring the course of these episodes.

Plasma exchange in severe erythema nodosum leprosum.

Four patients with severe erythema nodosum leprosum were treated by plasma exchange and/or fresh frozen plasma infusions after failure of classical therapy. After the procedures, the patients improved rapidly; with a follow-up between 4 and 7 years after the last plasma exchange, no clinical relapse was noted. The replacement fluids were variable; the most beneficial procedure seemed to be plasma exchange replaced with fresh frozen plasma. Elimination of circulating immune complexes, replacement of a lacking plasma factor are possible mechanisms of action. Plasma exchange may also work like a regulator of immune mechanisms, since it has been shown that there is a depression of suppressor cells in erythema nodosum leprosum.

[Necrotic course of erythema nodosum leprosum]. / Erythema nodosum leprosum à évolution nécrotique.

During 2 years, 25 lepromatous patients were hospitalised in the Hansen complications room of Institut Marchoux. Between these patients, 9 developed a Necrotic Erythema Nodosum Leprosum. A review of the observed clinic effects is established and 3 types of signs are isolated and discussed: necrotic extension after big nodes on chest and arms, a punch crater complicating small nodes, and a sclerous xylodermia on arms and legs. The course of this complication is estimated about six months average, with pauses and relapses with general and subjectives symptoms. The final course shows side effects: anemia, denutrition, functional disabilities of joint movements and cutaneous straps. In the group of 9 patients, 3 died. We did not find relations between the necrotic phenomenon and therapy or occurred diseases. The best drug to stop the necrotic processus is thalidomide 400 mg daily and decrease 100 mg when the signs fall near normality. One or two mg/kg each day coricosteroides were tested: the effect is unconstant, the duration of action is shorter. Side effects occur rapidly and patient will become corticosteroïd dependent. The importance of bath with disinfectants is high. We did not observed surinfection.

Treatment of leprosy with weekly intravenous infusion of leukocytes.

Two patients with lepromatous leprosy were treated with weekly intra-venous infusions of leukocyte concentrates for a period of 12 consecutive weeks. A reversal reaction was induced in one of the patients, and it was possible to control chronic erythema nodosum leprosum in the other subject. Possible pathogenetic mechanisms involved in the induction of these changes include the action of transfer factor, interactions between B- and T-lymphocytes or the mediation of a lymphokine necessary for the effective function of the cell-mediated immunity. Immunotherapy for chronic infections, such as leprosy, still has not become a reality.