Lupus eritematoso cutáneo y lupus eritematoso sistémico: revisión de una historia sin fin / Cutaneous lupus erythematosus and systemic lupus erythematosus: endless story review

l lupus eritematoso es una afección que se conoce desde antiguo, referida en especial al compromiso cutáneo, pero ha sido mejor definida desde principios del siglo XIX, constituyéndose como una enfermedad sistémica con una base autoinmune. Mecanismos patogénicos inmunogenéticos desempeñan un papel en la susceptibilidad a la enfermedad, sobre la que actuarían variaciones hormonales y factores ambientales. De esta interacción deriva la producción de múltiples anormalidades inmunológicas, cuya acción sobre los tejidos da origen a la expresión clínica de la enfermedad. La relación entre el compromiso cutáneo, en especial crónico discoide, y sistémico, ha sido un factor de polémica, importante de dilucidar por el significado terapéutico y de pronóstico para el enfermo. De esta revisión se puede concluir que las diferentes manifestaciones del lupus constituyen una misma enfermedad, pero probablemente factores etiopatogénicos genéticos, hormonales y ambientales marcan la diferencia.

Lupus erythematosus is a condition that has been known since ancient times, especially referring to skin involvement, but has been better defined since the beginning of the 19th century, constituting itself as a systemic disease with an autoimmune basis. Immunogenetic pathogenic mechanisms play a role in disease susceptibility, on which hormonal variations and environmental factors would act. From this interaction derives the production of multiple immunological abnormalities, whose action on the tissues gives rise to the clinical expression of the disease. The relationship between cutaneous involvement, especially chronic discoid, and systemic, has been a controversial factor, important to elucidate due to its therapeutic and prognostic significance for the patient. From this review it can be concluded that the different manifestations of lupus constitute the same disease, but genetics, hormonal and environmental etiopathogenic factors probably make the difference.

Lupus tumidus: a report of two cases

Abstract Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous-violaceous lesions that leave no scars after regression. Histopathology reveals perivascular and periannexal lymphohistiocytic infiltrates in the papillary and reticular dermis and interstitial mucin deposition. Treatment is based on photoprotection, topical corticosteroids and antimalarials. We report two cases of lupus tumidus, which deserve attention for their low frequency in the literature, in addition to their relevance as a differential diagnosis among dermatologic disorders.

Linear cutaneous lupus erythematosus following the lines of Blaschko - Case report

Abstract: Chronic cutaneous lupus erythematosus in a linear configuration is rare, particularly in children, demonstrating similar incidence in both genders, no photo-sensitivity and lower probability of progression to systemic disease. We describe the case of a 9-year-old girl who presented erythematous papules with central atrophy on the upper and lower right limbs, asymptomatic and following the lines of Blaschko, since age four. Histological examination showed atrophy of the epidermis with aggression from epidermal-dermal interface and periadnexal and perivascular lymphocytic inflammatory infiltrate. Laboratory tests showed ANA in a titer of 1:320, in a dense and fine speckled pattern. Due to the rarity of presentation and location of the disease, this case is reported here.

Modelo estadístico para predecir el puntaje de desarrollo psicomotor de niños de 4 a 5 años de edad en función del nivel socioeconómico: Proposal of a statistical model to predict the score of psychomotor development of children 4 to 5 years according to socioeconomic level / Proposal of a statistical model to predict the score of psychomotor development of children 4 to 5 years according to socioeconomic level

Introducción: estudios recientes enfatizan la importancia de examinar el efecto del nivel socioeconómico sobre el desarrollo psicomotor infantil, en tal sentido las investigaciones informan que el nivel económico y el ambiente sociocultural en que se desenvuelve el niño son fundamentales para favorecer un normal desarrollo en la infancia Objetivos: proponer un modelo estadístico para predecir el puntaje de desarrollo psicomotor de niños y niñas de 4 a 5 años de edad en función del nivel socioeconómico. Metodología: se evaluó el desarrollo psicomotor a una muestra de 228 niños y niñas por medio de la aplicación de la batería TEPSI. Para determinar el nivel socioeconómico se aplicó una encuesta sociodemográfica a los padres y apoderados. Se efectuó un análisis correlacional bivariado no paramétrico, además se realizaron pruebas ANOVA de un factor, finalmente para modelar se efectuó un análisis de regresión múltiple. Resultados: los resultados reportaron que el nivel educacional del jefe de hogar y el ingreso per cápita correlacionan significativamente con el desarrollo psicomotor. Conclusiones: en cuanto a los modelos, se informa que fue posible diseñar tres modelos. El primero permite predecir un 4,2% del puntaje en el desarrollo psicomotor en función de la variable ingreso per cápita. El segundo modelo es capaz de predecir en un 11% el puntaje del lenguaje en base al variable ingreso per cápita. Por último, el tercer modelo, con bajo valor predictor (2,2%), permite predecir el puntaje de la coordinación en función del nivel educacional del jefe de hogar.

Introduction: recent studies emphasize the importance of examining the effect of socioeconomic status on child psychomotor development. In this sense, research conducted reports that the level of economic and cultural environment child operate in, are crucial for facilitating their normal development. Objectives: the study proposes a statistical model to predict the psychomotor development score of children from 4 to 5 years of age depending on the socio-economic level. Methodology: psychomotor development was evaluated in a sample made up of 228 boys and girls through the application of the psychomotor development test (TEPSI). A demographic survey was applied to parents and guardians to determine their socio-economic level. A non-parametric bivariate correlational analysis was performed, and a single factor ANOVA tests were carried out. Last, a multiple regression analysis was made for modelling purposes. Results: the results reported that the educational level of the head of household and the per capita income is significantly correlated with the psychomotor development. Conclusions: as to models, the possibility of designing three models is reported. The first one allows predicting 4.2 % of the psychomotor development score based on the per capita income variable. The second model is able to predict 11% of the language score based on the per capita income variable. Last, the third model, with low predictive value (2.2 %), enables predicting the coordination score, based on the educational level of the head of household.

Lupus eritematoso neonatal: reporte de un caso / Neonatal lupus erythematosus: case report

El lupus eritematoso neonatal (LEN) es una enfermedad muy poco frecuente que se presenta en el recién nacido, dada por el pasaje transplacentario de autoanticuerpos anti Ro/SSA, anti La/SSB y U1-RNP. Las principales manifestaciones clínicas son dermatológicas y cardíacas, pudiendo además presentar manifestaciones hepáticas, hematológicas y/o neurológicas. Presentamos un caso clínico de un lactante de 3 meses al que se le realizó diagnóstico de LEN en base a la presencia de lesiones cutáneas y hallazgos histopatológicos e inmunológicos compatibles, no se acompañó de manifestaciones sistémicas y presentó una muy buena evolución posterior. El interés del caso es mostrar una patología poco frecuente que suele presentarse con lesiones cutáneas características, destacando que el pronóstico estará determinado por el compromiso cardíaco y que permite en muchos casos realizar diagnóstico materno de una enfermedad autoinmune asintomática.

Neonatal lupus erythematosus (LEN) is a rare disease that occurs in the newborn given by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and Anti-U1-RNP autoantibodies. The main clinical manifestations are dermatologic and cardiac, it may also have hepatic, hematologic or/and neurological ones. The study reports a case of a three month infant who is diagnosed with LEN, based on the presence of consistent findings of skin lesions and histopathology and immunology compatible findings. In this case, it was not accompanied by systemic manifestations evolution was a favorable one. The case is relevant since it presents an unusual condition that is usually accompanied by characteristic skin lesions, where prognosis is determined by cardiac involvement. In many cases, this enables maternal diagnosis of an asymptomatic autoimmune disease.

Anti-TNF-α and hydralazine drug-induced lupus

Drug-induced lupus is a rare drug reaction featuring the same symptoms as idiopathic lupus erythematosus. Recently, with the introduction of new medicines in clinical practice, an increase in the number of illness-triggering implicated drugs has been reported, with special emphasis on anti-TNF-α drugs. In the up-to-date list, almost one hundred medications have been associated with the occurrence of drug-induced lupus. The authors present two case reports of the illness induced respectively by hydralazine and infliximab, addressing the clinical and laboratorial characteristics, diagnosis, and treatment.

Drug-induced lupus with leukocytoclastic vasculitis: a rare expression associated with adalimumab

TNF alpha antagonist-induced lupus-like syndrome is a rare condition which predominantly affects women (4:1). The average age of onset is 46-51 years. It occurs after exposure to TNF alpha antagonist and disappears after discontinuation of such agents. The pathogenic mechanism for development of the TNF alpha antagonist-induced lupus-like syndrome is not fully defined. It is believed that the medication induces apoptosis, leading to an accumulation of nucleosomal antigens of apoptotic cells. This would cause autoantibodies to be produced by susceptible individuals. The most common cutaneous manifestations include maculopapular exanthem, malar rash, alopecia, photosensitivity and, more rarely, vasculitis. Extracutaneous manifestations include: fever, weight loss, arthritis or arthralgia, myositis and hematological abnormalities. Antinuclear antibody may be positive in 80% of cases and anti-histone antibody is considered a disease marker for TNF alpha antagonist-induced lupus-like syndrome. Treatment corresponds to drug discontinuation. We report a rare case of sub-acute cutaneous lupus erythematosus with leukocytoclastic vasculitis induced by adalimumab in a 42-year-old patient.

Lupus erythematosus: considerations about clinical, cutaneous and therapeutic aspects

Systemic Lupus Erythematosus is a chronic inflammatory disease with multifactorial etiology. Although clinical manifestations are varied, the skin is an important target-organ, which contributes to the inclusion of skin lesions in 4 out of the 17 new criteria for the diagnosis of the disease, according to the Systemic Lupus International Collaborating Clinics. The cutaneous manifestations of lupus are pleomorphic. Depending on their clinical characteristics, they can be classified into Acute Cutaneous Lupus Erythematosus, Subacute Cutaneous Lupus Erythematosus, Chronic Cutaneous Lupus Erythematosus and Intermittent Cutaneous Lupus Erythematosus. Treatment is based on preventive measures, reversal of inflammation, prevention of damage to target organs and relief of adverse events due to pharmacological therapy. The most commonly used treatment options are topical, systemic and surgical treatment, as well as phototherapy. The correct handling of the cases depends on a careful evaluation of the morphology of the lesions and the patient's general status, always taking into consideration not only the benefits but also the side effects of each therapeutic proposal.

Tinea Incognito in Korea and Its Risk Factors: Nine-Year Multicenter Survey

Tinea incognito (TI) is a dermatophytic infection which has lost its typical clinical appearance because of improper use of steroids or calcineurin inhibitors. The incidence of TI is increasing nowadays. We conducted retrospective review on 283 patients with TI from 25 dermatology training hospitals in Korea from 2002-2010 to investigate the demographical, clinical, and mycological characteristics of TI, and to determine the associated risk factors. More than half (59.3%) patients were previously treated by non-dermatologists or self-treated. The mean duration of TI was 15.0 +/- 25.3 months. The most common clinical manifestations were eczema-like lesion, psoriasis-like, and lupus erythematosus-like lesion. The trunk and face were frequently involved, and 91 patients (32.2%) also had coexisting fungal infections. Among 67 isolated strains, Trichophyton rubrum was the most frequently detected (73.1%). This is the largest study of TI reported to date and the first investigational report concerning TI in Korea. We suggest that doctors should consider TI when a patient has intractable eczema-like lesions accompanied by tinea pedis/unguium. Furthermore, there should be a policy change, which would make over-the-counter high-potency topical steroids less accessible in some countries, including Korea.

Lupus eritematoso neonatal: reporte de cuatro casos / Neonatal lupus erythematosus: a report of four cases

El lupus eritematoso neonatal es una enfermedad poco frecuente, caracterizada clínica mente por alteraciones cutáneas semejantes al lupus subagudo o discoide y/o bloqueo cardíaco congénito. Generalmente, cuando los pacientes presentan manifestaciones cutáneas, no tienen anormalidades cardiológicas y viceversa, aunque en un 10 por ciento de los casos ambas manifestaciones pueden coexistir. Puede acompañarse también de alteraciones hematológicas, hepáticas y neurológicas. Es causado por el pasaje trasplacentario de anticuerpos maternos anti Ro (95 por ciento), anti La y menos frecuentemente anti U1RNP. Presentamos cuatro pacientes con hallazgos clínicos, histopatológicos e inmunológicos compatibles con lupus eritematoso neonatal, su tratamiento y evolución.

Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10 percent of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95 percent), La and, less frequently, U1-ribonucleoprotein (U1-RNP). The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress.

Imunofluorescência direta e indireta / Direct and indirect immunofluorescence

A imunofluorescência é um valioso instrumento auxiliar no diagnóstico das dermatoses bolhosas autoimunes e desordens inflamatórias, uma vez que seus achados clínicos e histopatológicos podem não ser determinantes. Consiste em um método laboratorial factível, que requer profissionais técnicos experientes, e detecta imunocomplexos in situ e/ou circulantes, que podem estar envolvidos na patogênese de tais enfermidades cutâneas.

Immunofluorescence is a valuable auxiliary diagnostic tool for autoimmune bullous diseases and inflammatory disorders, since their clinical and histopathologic findings may be inconclusive. It is a feasible laboratory method that requires experienced technicians and detects in situ and circulating immune deposits that may be involved in the pathogenesis of such skin diseases.

Lupus eritematoso cutáneo subagudo (LECSA): a propósito de 17 casos con anticuerpo anti-Ro positivo / Subacute cutaneous lupus erythematosus (SCLE): 17 patients with anti-Ro antibodies

Se presentan, analizan y comparan con otras series los hallazgos dermatológicos, clínicos, histopatológicos e inmunológicos de 17 casos de lupus eritematoso cutáneo subagudo (LECSA) con anticuerpos (Ac) anti-Ro positivos.Las manifestaciones dermatológicas de nuestros pacientes fueron lesiones papuloescamosas policíclicas, más frecuentes que las psoriasiformes. Se localizaban preferentemente en miembros superiores, dorso y zonas de exposición sola. Presentaban un borde eritematovesiculoso bien delimitado. Otras lesiones adoptaron la forma de reloj de arena. Estas manifestaciones fueron recurrentes con períodos de actividad y calma. También fueron relevantes la fotosensibilidad, la hipopigmentación y, en algunos casos, discreta atrofia. Histopatológicamente lo más destacable fue la degeneración vacuolar de la capa basal de queratinocitos y la ausencia de hiperqueratosis folicular, diferenciándose del lupus eritematoso discoide crónico (LEDC). Las restantes lesiones se parecían a las del lupus eritematosos sistémico (LES), pero con menor intensidad. Hubo poco engrosamiento de la menbrana basal PAS positiva, y el infiltrado linfocitario subpapilar y perivascular fue escaso. En algunos casos se demostró edema dérmico alcian blue positivo, poniendo de manifiesto la presencia de mucina. A diferencia de lo relatado por otros autores, encontramos discreta atrofia en 4 pacientes. Lo más significativo de los exámenes inmunológicos fue la presencia del Ac anti-Ro en el 100% de los enfermos y el anti-La solo en el 17,6%. Fue criterio de inclusión para esta serie tener anti-Ro positivo con anti-ADN y anti-Sm negativos. El FAN fue positivo en el 70%. Con respecto a las manifestaciones clínicas generales, se observaron artritis/artralgias en el 100% de los casos. Las lesiones fueron simétricas, no erosivas ni deformantes. Un solo enfermo tuvo pleuresia y glomerulonefritis crónica difusa...

Clinical, histopathologic, and immunological findings in 17 patients with subacute cutaneous lupus erythematosus (SCLE) and Roantibodies are described. Skin manifestations consisted of recurrent, polycyclical, circumscribed papulosquamous, psoriasiform, or hourglass-like lesions with vesiculoerythematous borders, mainly on the upper limbs, back, and sun-exposed areas. Photosensitivity, hypopigmentation, and occasionally mild atrophy were also noted. Histopathologic features included vacuolar degenerative changes involving keratinocyte basement membrane. Unlike chronic discoid lupus erythematosus lesions, follicular hyperkeratosis was absent. Although other characteristics were similar to those seen in systemic lupus erythematosus patients, specimens from SCLE showed less basement membrane thickening and inflammatory cell infiltrates. A few samples revealed positive alcian blue staining for dermal mucin. Only four patients had moderateatrophy. Since only Ro-positive, and DNA- and Sm-negative patients were assessed, immunological studies showed Ro antibodies in 100%of cases and La antibodies in 17.6%. FAN measurements using rat liver and Hep-2 cells were positive in 70% of patients. SCLE was associated with symmetrical, non-erosive, and non-deforming arthritis/arthralgia in all patients, vasculitis in three (17.8%), Raynaud’s syndrome in two (11.8%), pleuritis and chronic glomerulonephritis in one, and panniculitis in one. Ro antibody screening tests were reviewed.

Estudo de lesões orais associadas a doenças dermatologicas / Study of oral lesions associated to dermatologic diseases

No presente trabalho, nos propusemos a avaliar a freqüência das lesões orais em pacientes portadores de doenças dermatológicas, visando despertar o cirurgião dentista para a necessidade de estabelecer um diagnóstico preciso. A amostra do nosso estudo constou de 101 pacientes procedentes do Serviço de Dermatologia do Hospital Universitário Onofre Lopes (HUOL) da UFRN com dermatopatologias diagnosticadas os quais foram examinados clinicamente com intuito de identificar alterações bucais. A idade dos pacientes variou entre 20 e 60 anos de idade, havendo predileção pelo sexo feminino (1.8:1). Do total de casos avaliados, 43 porcento eram de lúpus eritematoso, 35 porcento de líquen plano, 11 porcento de pênfigo vulgar, 8 porcento de eritema multiforme e 3 porcento eram do grupo penfigóide. As manifestações orais, em valores absolutos, foram mais freqüentes nos pacientes portadores de líquen plano (17) seguido pelo pênfigo (3), grupo penfigóide (2) e lúpus eritematoso (2). O líquen plano reticular foi a apresentação clínica mais comum e a mucosa jugal o sítio mais acometido. Os portadores de pênfigo e grupo penfigóide exibiram preferencialmente lesões de aspecto ulcerativo distribuídas na região de mucosa jugal e orofaringe, enquanto o lúpus eritematoso apresentava lesões circunscritas e eritematosas. Não foram observadas alterações bucais nos casos de eritema multiforme. De acordo com os resultados obtidos concluímos que o líquen plano foi a dermatopatologia mais prevalente em nosso estudo em relação à freqüência das alterações orais apresentadas, a partir da qual podemos inferir a necessidade do conhecimento do cirurgião dentista neste contexto e a importância do seu papel para que seja estabelecido um diagnóstico precoce e orientado o tratamento adequado com uma abordagem multidisciplinar

Lupus eritematoso neonatal: presentación de un caso y revisión de la literatura / Neonatal lupus: case report and literature review

El lupus eritematoso neonatal es un síndrome muy poco frecuente caracterizado por alteraciones cutáneas semejantes al lupus subagudo o discoide, acompañado frecuentemente por alteraciones cardíacas, en especial trastornos de la conducción, y alteraciones sistémicas. Se debe al pasaje pasivo transplacentario de anticuerpos antiRo y antiLa de la madre, quien padece o padecerá enfermedades relacionadas como lupus eritematoso sistémico, síndrome de Sjogren u otras enfermedades reumáticas. Presentamos el primer caso argentino conocido, en un lactante varón cuya madre y abuela materna padecían lupus eritematoso sistémico. Sus manifestaciones cutáneas, conformadas por máculo-pápulas eritematosas figuradas de cara y miembros y petequias en tronco, fueron desvaneciéndose con el correr de los meses. El niño se encontraba libre de lesiones cutáneas a los 6 meses de vida. La microscopía óptica y la inmunofluorescencia directa confirmaron el diagnóstico, siendo negativos los estudios de anticuerpos circulantes antiRo, antiLa, antiADN y antiRNP y positivo débil el FAN