Porphyria cutanea tarda and systemic lupus erythematosus.

The co-existence of systemic lupus erythematosus and porphyria although rare has been known for a long time. This association forces the physician to make a careful differential diagnosis of the bullous lesions that might appear in such patients and to be careful when prescribing certain drugs such as chloroquine. This drug, when used in the regular doses for treating lupus, may cause hepatotoxicity in patients.suffering from porphyria. It is described here the case of a patient with lupus who developed bullous lesions compatible with porphyria cutanea tarda.

Liver autoantibodies in patients with scleroderma.

Association between autoimmune liver diseases and scleroderma has been described. The purpose of this study was to study the prevalence of antimithocondrial antibody (AMA), antismooth muscle antibodies (SMA), and liver-kidney-microsomal (LKM-1) autoantibody in a cohort of 63 scleroderma patients and 100 healthy controls. The autoantibodies AMA, SMA, and LKM were determined by indirect immunofluorescence. Patients' charts were reviewed for demographic data, scleroderma form, and clinical and anti-nuclear antibody profile, aiming a comparison between patients with and without liver autoantibodies. Nine patients (14.3%) were positive for at least one of the liver autoantibodies; only one patient had both AMA and SMA positive. Antibody SMA was positive in 6.4% (4/63) patients; AMA was present in 9.52% (6/63) of them; none were positive to LKM-1. In the control group just one patient (1%) was SMA positive; the other autoantibodies were negative. There is an increased prevalence of liver autoantibodies in patients with scleroderma than in control population. These patients should be carefully followed for liver dysfunction.

Porfiria cutânea tarda e lúpus eritematoso sistêmico / Porphyria cutanea tarda and systemic lupus erythematosus

A associação de lúpus eritematoso sistêmico e porfiria, embora rara, é conhecida de longa data. Ela obriga o médico a realizar um cuidadoso diagnóstico diferencial das lesões bolhosas nesses pacientes e tomar cuidados com a prescrição de certas drogas, como a cloroquina. Esta, nas doses habituais para tratamento do lúpus, pode causar hepatotoxicidade em pacientes com porfiria. Descreve-se o caso de uma paciente com lúpus que desenvolveu lesões bolhosas compatíveis com porfiria cutânea tardia.

The co-existence of systemic lupus erythematosus and porphyria although rare has been known for a long time. This association forces the physician to make a careful differential diagnosis of the bullous lesions that might appear in such patients and to be careful when prescribing certain drugs such as chloroquine. This drug, when used in the regular doses for treating lupus, may cause hepatotoxicity in patients.suffering from porphyria. It is described here the case of a patient with lupus who developed bullous lesions compatible with porphyria cutanea tarda.

Mal de Hansen, anticorpos antifosfolípides e obstrução das artérias fibulares / Leprosy, antiphospholipid antibodies and bilateral fibular arteries obstruction

Pacientes com Mal de Hansen (MH) podem se apresentar com quadro clínico e laboratorial sugestivo de doenças reumáticas, o que exige um exercício cuidadoso de diagnósticos diferenciais. Descreve-se aqui o caso de uma jovem com MH que se apresentou com lesões cutâneas sugestivas de vasculite, obstrução de vasos fibulares, FAN e anticorpos antifosfolípides positivos sem muitos estigmas da doença cutânea, ilustrando essa dificuldade.

Patients with Hansen's disease (HD) may present themselves with clinical and laboratorial features that resemble rheumatic disorders. This requires a careful exercise of differential diagnosis. We describe here a case of a young woman with HD that presented with vasculitic like lesions, fibular vessels obstruction, ANA and antiphospholipid antibodies without typical signs of cutaneous disease, illustrating this clinical difficulty.

The adult patient with polyarticular juvenile idiopathic arthritis / O adulto com artrite idiopática juvenil poliarticular

Crianças com a forma poliarticular da artrite idiopática juvenil (AIJ), ao entrarem na idade adulta, têm um quadro similar ao de pacientes com artrite reumatoide (AR) de início no adulto. No presente estudo comparam-se características clínicas e imunológicas desses dois grupos de pacientes. Para isso, foram estudados vinte adultos com AIJ poliarticular e cinquenta pacientes com AR (pareados para sexo e tempo de duração de doença), para presença de autoanticorpos, nódulos subcutâneos, síndrome de Sjõgren secundária, hipotireoidismo e para a determinação de índices funcionais e antropométricos. Encontraram-se, nesses dois grupos, características similares, exceto pela presença de fator reumatoide (menor no grupo de AIJ poliarticular; P = 0,026) e menor IMC nos pacientes com AIJ poliarticular (P < 0,001).

When children with polyarticular juvenile idiopathic arthritis (JIA) reach adulthood, they have a condition similar to that of patients with adult onset rheumatoid arthritis (RA). In the present study, the clinical and immunological characteristics of these two groups of patients are compared. The presence of autoantibodies, subcutaneous nodules, secondary Sjõgren syndrome, and hypothyroidism, was determined in 20 adult patients with polyarticular JIA and in 50 patients with RA (paired for gender and duration of the disease), as well as the determination of functional and anthropometric indexes. Both groups had similar characteristics, except for the presence of rheumatoid factor (lower in the polyarticular JIA group; P = 0.026) and lower BMI in patients with polyarticular JIA (P < 0.001).