RESUMEN
A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.
Asunto(s)
Enfermedad Mixta del Tejido Conjuntivo/complicaciones , Enfermedades Cutáneas Vasculares/complicaciones , Úlcera Cutánea/etiología , Adulto , Anticuerpos Antinucleares/análisis , Biopsia , Diagnóstico Diferencial , Femenino , Antígenos HLA-DR/inmunología , Antígeno HLA-DR4/inmunología , Cadenas HLA-DRB4 , Humanos , Enfermedad Mixta del Tejido Conjuntivo/inmunología , Enfermedad Mixta del Tejido Conjuntivo/patología , Recurrencia , Ribonucleoproteínas Nucleares Pequeñas/inmunología , Enfermedades Cutáneas Vasculares/etiología , Enfermedades Cutáneas Vasculares/inmunología , Enfermedades Cutáneas Vasculares/patología , Úlcera Cutánea/patologíaRESUMEN
Giant-cell interstitial Pneumonia (GIP) is a very uncommon respiratory disease. The majority of cases of GIP are caused by exposure to cobalt, tungsten and other hard metals. In this report, we describe GIP in a patient who worked in gas station and dealt in propane gas vessels. He presented with clinical features of chronic interstitial lung disease and underwent an open lung biopsy that showed DIP-like reaction with large numbers of intra-alveolar macrophages and numerous large, multinucleated histiocytes which were admixed with the macrophages. Analysis of lung tissue for hard metals was done. Cobalt was the main component of detected hard metals. Corticosteroid therapy was started and he recovered fully.
Asunto(s)
Enfermedades Pulmonares Intersticiales/diagnóstico , Neumonía/diagnóstico , Células Gigantes , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Enfermedades Pulmonares Intersticiales/patología , Masculino , Persona de Mediana Edad , Neumonía/diagnóstico por imagen , Neumonía/tratamiento farmacológico , Neumonía/patología , RadiografíaRESUMEN
We report a 25-year-old Korean woman with Adult onset Still's disease (AOSD) presented with renal amyloidosis, which had developed four years after disease onset. We successfully treated her with prednisolone, colchicine and cyclophosphamide. A review of the literature uncovered about 10 cases, most of which were treated by various regimens that resulted in poor outcomes. Renal amyloidosis should be suspected in patients with AOSD who have unexplained proteinuria. Although the mechanism of renal amyloid deposition is not well known, earlier histopathologic diagnosis and choice of regimen may affect prognosis.