Diffuse leprosy of Lucio and Latapí: a histologic study.

BACKGROUND AND PURPOSE: Ladislao de la Pascua described the spotted or lazarine leprosy for first time in 1844. Later on, Lucio and Alvarado studied and published it with the same names in 1852. Latapí re-discovered it in 1938 and reported it as 'Spotted' leprosy of Lucio in 1948. Frenken named it diffuse leprosy of Lucio and Latapí in 1963. Latapí and Chévez-Zamora explained that the fundamental condition of this variety of leprosy was a diffuse generalised cutaneous infiltration, naming it pure and primitive diffuse lepromatosis, upon which necrotising lesions develop, calling these lesions Fenómeno de Lucio or erythema necrotisans. A great number of histopathological reports have addressed the study of Lucio's phenomenon, and few about the histologic changes that take place in the course of diffuse lepromatous leprosy. The purpose of this work is to report the histologic findings observed in the study of 170 cutaneous biopsies of diffuse leprosy of Lucio and Latapí and 30 of Lucio's phenomenon. METHODS: This is a retrospective study, which included the examination of 200 biopsy skin specimens from 199 patients with diffuse leprosy at different course of the disease. These cases were diagnosed in Mexico from 1970 to 2004. RESULTS: The histologic examination revealed a vascular pattern affecting all cutaneous vessels, characterised by five outstanding features: a) colonisation of endothelial cells by acid-fast bacilli, b) endothelial proliferation and marked thickening of vessel walls to the point of obliteration, c) angiogenesis, d) vascular ectasia, and e) thrombosis. Necrotising lesions seen in diffuse lepromatous leprosy displayed two histopathological patterns: one of them, non-inflammatory occlusive vasculopathy and, the other one, occlusive vasculopathy, leukocytoclastic vasculitis, large neutrophilic infiltrate and lobular panniculitis. The first appeared as a result of the course of the occlusive vasculopathy produced by the colonisation of endothelial cells by Mycobacterium leprae. The second, as a result of a previous occlusive vasculopathy plus a leprosy reaction which is considered here as variant of ENL. CONCLUSIONS: Endothelial cell injury appears to be the main event in the pathogenesis of diffuse leprosy of Lucio and Latapí. Once M. leprae has entered the endothelial cell, the micro-organism damages the blood vessels, leading to the specific changes seen in this variety of lepromatous leprosy.

Eritema nodoso / Erythema Nodosum

Os autores fazem uma revisão bibliográfica sobre eritema nodoso, incluindo suas diversas causas, manifestações clínicas, investigação e diagnósticos diferenciais. É enfatizada que eritema nodoso pode ser a manifestação cutânea de uma doença sistêmica, logo, essa situação deve ser afastada

Pulsed corticosteroid therapy in patients with chronic recurrent ENL: a pilot study

A pilot study has been undertaken to compare the efficacy of small dose pulsed betamethasone therapy with need based oral steroids in chronic recurrent erythema nodosum leprosum (ENL) patients. Though this mode of therapy was well tolerated, no advantage with intermettnt steroid administration was observed. This coul have been on account of small dose of steroid given monthly. Treatment of chronic recurrent erythema nodosum leprosum (ENL) patients continues to be unsatisfactory, particularly, because of non-availability of thalidomide. Though corticosteroids are effective in suppressing all the manifestations and even restoring partially or fully the functional impairment, their side effects and dependence are equally troublesome. Based on (a) the reported efficacy and safety of intermittent use of corticosteroids in several immune complex mediated disorders (Cathcart et al 1976, Kimberly et al 1979), Lieblin et al 1981 and Pasricah & Gupta 1984) and (b) ENL (type II) reactions having similar pathology, a pilot study has been undertaken to see the efficacy and the tolerance of pulsed steroids in chronic ENL patients

Utility of fine-needle aspiration cytology in the classification of leprosy.

The role of fine-needle aspiration cytology (FNAC) in the diagnosis of benign skin lesions has been restricted primarily to the evaluation of bacteriologic and morphologic indices in leprosy. This study was undertaken to evaluate the efficacy of FNAC in the diagnosis and classification of lepromatous lesions. Aspirates of 94 newly diagnosed cases of leprosy were studied, and the bacterial load was determined by modified Ziehl-Neelsen (ZN) stain. A skin biopsy was taken from the same site at the same sitting. Frozen and paraffin sections stained with hematoxylin-eosin (H&E) and ZN stains were examined from the biopsy specimen. In 61 of 94 cases (64.9%), the aspirates were satisfactory. Both diagnosis and classification of leprosy were possible in 40 of these 61 cases; the rest of the aspirates showed nonspecific chronic inflammation. The 39 cases of leprosy where a biopsy was available from the same site were classified on FNAC into tuberculoid (TT and BT), lepromatous (LL and BL), and midborderline (BB) subtypes. Taking the histologic diagnosis and Ridley-Jopling classification to be the gold standard, a strong concordance in tuberculoid leprosy cases (18 of 20 cases, 90%) and in lepromatous cases (15 of 16 cases, 93.7%) was observed. Midborderline cases of leprosy posed a problem, and a correct cytohistological correlation was observed in only one of the three cases.

Evolución bacteriológica de la lepra lepromatosa según la presencia de episodios de eritema nudoso leproso

El ENL es una reacción frecuentemente observada en pacientes con lepra lepromatosa (LL), que parece desencadenarse poe le depósito de inmunocomplejos en la pared vascular y fenómenos de inmunidad celular, alguno de ellos específicos para el M. leprae. Para determinar si la presencia de ENL podía facilitar mejor evolución de la LL, evaluamos el tiempo de negativación bacteriológica (TNB) de pacientes LL según la aparición de episódios de ENL a lo largo del tratamiento. Se analisaron retrospectivamente las historias clínicas de 106 pacientes tratados mayormente con sulfas, 27 casos que nunca experimentaron episodios de ENL y 79 enfermos que lo habían desarrollado con distinta intensidad y frecuencia. Ambos grupos fueron similares en cuanto a edad, distribución por sexo, tipo de tratamiento y superficie corporal efectada, registrándose diferencias en la variedad clínica, más casos maculares en los LL sin ENL. Éstos tuvieron un TNB significativamente menos (3.1±0.4 años) al de los LL con ENL (6.1±0.3, media±es). Una subdivisión del último grupo según la magnitude y periodocidad de los episodios ENL tampoco reveló diferencias en el TNB, mostrando valores similares al registrado en el grupo original. La aparición de episódios de ENL no parece acelerar el tiempo de aclaramiento bacilar.

Paniculitis septal y eritema nudoso típico, su diferenciación sobre bases clínicas e histopatológicas / Panniculitis septal and typical erythema nodosam, clinical and histopathologic differentiation

Se estudiaron 11 pacientes, siete con diagnóstico de Eritema Nudoso y cuatro con diagnóstico de Paniculitis Nodular Subaguda Migratoria. Se establecieron diferencias entre ambas patologías sobre bases clínicas e histopatológicas. El Eritema Nudoso Típico se caracterizó por presenta capilaritis, flebitis y hemorragia a nivel de septum y lóbulos adiposos, e infiltrado linfo-hitiocitario perivascular. La Paniculitis Subaguda Nodular Migratoria presentó marcada fibrosis septal, proliferación capilar perilobular adiposa e intensa reacción granulomatosa con células gigantes de cuerpo extraño a lo largo del borde del séptum interlobular. Los Lóbulos adiposos mostraron atrofia, con mínimo cambios inflamatorios. Llegamos a la conclusión que existen suficientes hallazgos clínicos e histopatológicos que justifican considerar a la Paniculitis Septal Migratoria y al Eritema Nudoso Típico entidades diferentes

Paniculitis septal y eritema nudoso típico, su diferenciación sobre bases clínicas e histopatológicas / Panniculitis septal and typical erythema nodosam, clinical and histopathologic differentiation

Se estudiaron 11 pacientes, siete con diagnóstico de Eritema Nudoso y cuatro con diagnóstico de Paniculitis Nodular Subaguda Migratoria. Se establecieron diferencias entre ambas patologías sobre bases clínicas e histopatológicas. El Eritema Nudoso Típico se caracterizó por presenta capilaritis, flebitis y hemorragia a nivel de septum y lóbulos adiposos, e infiltrado linfo-hitiocitario perivascular. La Paniculitis Subaguda Nodular Migratoria presentó marcada fibrosis septal, proliferación capilar perilobular adiposa e intensa reacción granulomatosa con células gigantes de cuerpo extraño a lo largo del borde del séptum interlobular. Los Lóbulos adiposos mostraron atrofia, con mínimo cambios inflamatorios. Llegamos a la conclusión que existen suficientes hallazgos clínicos e histopatológicos que justifican considerar a la Paniculitis Septal Migratoria y al Eritema Nudoso Típico entidades diferentes

ENL. quantification - a proposal

The authors propose a method of quantification of type II reactions, which will give to the clinicians and research workers common parameters for evaluatin the severity of a reactions as well as the criteria dor assessing the efficacy of a given drug.

[Erythema nodosum in childhood. Nosographical classification and case studies]. / L'eritema nodoso nell'infanzia. Inquadramento nosografico e contributo casistico.

A survey of the relevant literature, with particular attention to clinical and immunological studies propounding pathogenetic theories, is followed by the presentation of 32 cases of erythema nodosum observed in infants examined at the Turin Paediatrics Clinic and Regina Margherita Hospital. The clinical laboratory data showed that streptococcal infection was hardly less significant than T. B. as the cause of this condition.

[Reactional status of leprosy]. / Estados reacionais da lepra

Reactional leprosy is studied according to its clinical forms A) Lepromatous a) Acute lepromatization: encroaching and invasive nature; the patient becomes more and more lepromatous ; bad prognosis. b) Erythema nodosum: "contusiform dermatitis"; variable prognosis not so bad as it is in the preceding case; allergic nature and its evolution is usually detained and therapeutics efficient. c) Erythema multiform. d) Lucio's phenomenon: vascular lesions and consequently necrosis as a complication of the "erythema necrotisans" (beautiful leprosy). B) Tuberculoid Reactional tuberculoid is the only one in this benign type, the Mitsuda's test must always be positive and prognosis consequently good. C) Dimorphous or "Borderline" whose Mitsuda's test is mostly negative, sometimes positive, but not stable. The lesions may stimulate the tuberculoid leprids but they invade mucous membranes, are impregnated by pigmentation, may present the Unna's band, and other characteristics of the Lepromatous type. Are associated (fever, asthenia and emaciation). Prognosis not very good, because of the possibility of lepromatization, according to its tendency. Evolution slower and frequent relapses. Besides there are nodular lesions. Pathogeny 1) Perifocal allergic reaction (Jadassohn). Similar to epituberculosis and Herxheimer reaction. 2) Septicemia. Sensitized tissues inside or outside the lesions, are invaded by the bacilli and so the allergic reaction takes place. Even without culture resources, Mycobacterium leprae has been found in the blood by direct examination. 3) Autoimmunization (Waldenstrom, Matthews and Trantman, 1965). Based upon the similarity between both humoral syndromes, in leprosy reactions and collagenous, diseases, as to: hypergammaglobulins, hypercryoproteins, antigammaglobulins, serological reactions (Wassermann, Kahn, Kline, VDRL) positives, Antistreptolysin O, protein C reactive, antinuclear factors, latex and Wadler-Rose test positives (rheumatoid tests) lowering of complement. If leprosy reaction is like this, it should be the less agressive of the autoimmune diseases. a) Its eruptions are cyclic not of long standing duration, as a general rule. b) Its prognosis has been recognized as good, except lately, because of the use of corticoid therapy which has been fatal, in many cases. After some years the leprosy reaction cures spontaneously. Treatment (see article)