RESUMO
Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening dermatological conditions. Their most common cause is medication. However, in a small proportion of patients these dermatological conditions could be the first presentation of systemic lupus erythematosus. We now describe a 34-year-old patient who presented with manifestations of Stevens-Johnson as a first feature of systemic lupus erythematosus. Systemic lupus erythematosus reveled by Stevens-Johnson syndrome has been infrequently reviewed in the previous literature. This diagnosis should be considered when cutaneous adverse drug reactions occur without clear drug causality.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Síndrome de Stevens-Johnson/etiologia , Adulto , Anemia/etiologia , Artralgia/etiologia , Autoanticorpos/sangue , Complemento C3/análise , Cuidados Críticos , Dermatoses Faciais/etiologia , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Imunoglobulina G/análise , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Metilprednisolona/uso terapêutico , Mucosite/etiologia , Prednisona/uso terapêutico , Síndrome de Stevens-Johnson/tratamento farmacológicoRESUMO
BACKGROUND: Behçet's disease is a multisystemic inflammatory disease characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The diagnosis of Behçet's disease is based on clinical criteria. The etiology of the disease is unknown but the wide variations of ethnic prevalence and of the prevalence in the same ethnic group in different geographic areas indicate environmental triggering of a genetically determined disorder. PATIENTS AND METHODS: A retrospective analysis of the medical charts of 150 Behçet's disease patients seen in our internal medicine department between 1995 and 2010 was undertaken. Patients with confirmed ocular involvement were analyzed and compared with those without ocular involvement. RESULTS: Among the 150 medical charts studied, 85 patients were included in the study. Thirty-three patients (38.5%) had ocular involvement. Mean age at ocular BD diagnosis onset were 35.3. Male to female ratio was 5.6. Ocular involvement was bilateral in 26 patients (78.8%). Uveitis was the most common ocular lesion (n=31 patients, 93.9%). Panuveitis was the most common anatomical location (n=21, 63.6%). The comparison of patients treated for BD with or without ocular involvement showed a statistically significant association between ocular and neurological manifestations (p=0.03). All patients with ocular involvement were treated with corticosteroids. Immunosuppressive (IS) treatments were used in 28 patients (84.8%). Cyclophosphamide was the most used as first-line treatment (71.4%). Cyclophosphamide relayed by azathioprine was the most adopted protocol (28.5%). In case of resistance or relapse and depending on the other manifestations of the BD, the IS used in first intention was replaced by another one. Seven of the 33 patients had received treatment with infliximab (IFX) after failure of other therapeutic lines. CONCLUSION: Ocular prognosis in the BD can be improved by early treatment and regular monitoring. It is important to adjust the therapeutic protocol to the anatomic form, to the severity of uveitis and to the extra-ocular manifestations associated.
Assuntos
Síndrome de Behçet/epidemiologia , Oftalmopatias/epidemiologia , Adolescente , Adulto , Idoso , Síndrome de Behçet/complicações , Síndrome de Behçet/diagnóstico , Oftalmopatias/diagnóstico , Oftalmopatias/etiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Prognóstico , Estudos Retrospectivos , Tunísia/epidemiologia , Adulto JovemRESUMO
OBJECTIVE: To study the maternal and fetal outcomes in women with systemic lupus erythematosus. PATIENTS AND METHODS: A retrospective study of 26 pregnancies in 15 systemic erythematosus patients diagnosed before or during pregnancy regarding to American College of Rheumatology criteria in a single reference center. RESULTS: The mean patient age was 31.52 years (24-39 years). The mean interval from the diagnosis of the systemic lupus erythematosus to pregnancy was 4.2 years. Eight pregnancies were planned. The flare rate of lupus during pregnancy was 31%, life birth rate was 65% and fetal loss rate was 35%. DISCUSSION AND CONCLUSION: As an increase in disease activity can occur during pregnancy and because of a higher rate of obstetrical complications in patients with lupus, it is important to carefully plan pregnancy. Pregnancy in lupus patients must be closely monitored by a multispeciality care of the patients.