Malignant atrophic papulosis (Degos’disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement. We describe a 33-year-old man who presented with ...Malignant atrophic papulosis (Degos’disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement. We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. During the course of the disease he even developed penile ulcerations, a symptom that has been reported only a few times previously. He subsequently died of multiple perforations of the small bowel 2.5 years after onset of the disease. Laboratory investigations revealed a mutation of factor V Leiden and the presence of lupus anticoagulant, but no anti-cardiolipin antibodies. The patient was treated with narrow-band ultraviolet (UV)B, prednisolone and, later, aspirin, pentoxifyllin and warfarin. Despite this very intensive anti-coagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement.展开更多
Low factor V and VII levels are bad prognostic indicators in fulminant hepatic failure (FHF). The prognostic importance of admission versus follow up levels of these factors in patients with acute hepatitis and coagul...Low factor V and VII levels are bad prognostic indicators in fulminant hepatic failure (FHF). The prognostic importance of admission versus follow up levels of these factors in patients with acute hepatitis and coagulopathy without encephalopathy has not been evaluated. Clinical and laboratory data from 68 consecutive patients with acute hepatitis and coagulopathy but without encephalopathy, during a 6-year period, was retrospectively evaluated. Sixty patients (88%) demonstrated improvement in liver function and coagulation (‘survivors’), while 8 patients (12%) died or underwent OLT (‘non-survivors’). Survivors had higher admission (P < 0.005) and follow up factor VII levels (P < 0.005) than non-survivors. Follow up factor V levels were higher in survivors (P < 0.02), while admission factor V level was not different between groups (P=NS). Multivariate logistic regression analysis demonstrated that admission factor VII levels predicted outcome (P < 0.006). Area under the ROC curve of factor VII was larger than that of factor V (0.885 and 0.715, respectively, P < 0.02). After 3 days of hospitalization, factor V levels, but not factor VII, independently predicted outcome (P < 0.04). In patients with hepatitis and coagulopathy without encephalopathy at presentation, admission factor VII level may serve as a reliable prognostic marker. Subsequently, during hospitalization, changes in factor V are better outcome indicators.展开更多
文摘Malignant atrophic papulosis (Degos’disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement. We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. During the course of the disease he even developed penile ulcerations, a symptom that has been reported only a few times previously. He subsequently died of multiple perforations of the small bowel 2.5 years after onset of the disease. Laboratory investigations revealed a mutation of factor V Leiden and the presence of lupus anticoagulant, but no anti-cardiolipin antibodies. The patient was treated with narrow-band ultraviolet (UV)B, prednisolone and, later, aspirin, pentoxifyllin and warfarin. Despite this very intensive anti-coagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement.
文摘Low factor V and VII levels are bad prognostic indicators in fulminant hepatic failure (FHF). The prognostic importance of admission versus follow up levels of these factors in patients with acute hepatitis and coagulopathy without encephalopathy has not been evaluated. Clinical and laboratory data from 68 consecutive patients with acute hepatitis and coagulopathy but without encephalopathy, during a 6-year period, was retrospectively evaluated. Sixty patients (88%) demonstrated improvement in liver function and coagulation (‘survivors’), while 8 patients (12%) died or underwent OLT (‘non-survivors’). Survivors had higher admission (P < 0.005) and follow up factor VII levels (P < 0.005) than non-survivors. Follow up factor V levels were higher in survivors (P < 0.02), while admission factor V level was not different between groups (P=NS). Multivariate logistic regression analysis demonstrated that admission factor VII levels predicted outcome (P < 0.006). Area under the ROC curve of factor VII was larger than that of factor V (0.885 and 0.715, respectively, P < 0.02). After 3 days of hospitalization, factor V levels, but not factor VII, independently predicted outcome (P < 0.04). In patients with hepatitis and coagulopathy without encephalopathy at presentation, admission factor VII level may serve as a reliable prognostic marker. Subsequently, during hospitalization, changes in factor V are better outcome indicators.
