Pure red cell aplasia in a Sjogren's syndrome/lupus erythematosus overlap patient

Hematological complications of systemic lupus erythematosus usually include anemia of chronic disease and peripheral destruction of blood cells. We describe the case of a Sjogren’s syndrome/lupus erythematosus overlap, mother of infant young woman with congenital heart block, complicated by pure red cell aplasia. The patient was asymptomatic until the onset of severe anemia. A serum inhibitor of erythropoiesis was detected before the onset of immunosuppression. Bone marrow examination showed a low CD4:CD8 ratio, an immune defect possibly linked with the unrestrained production of antibodies against erythroid progenitor cells. (C) 2003 Wiley-Liss, Inc

Cutaneous ulcers: An unusual manifestation of inherited thrombophilia

We report two cases with cutaneous lesions found to be associated with factor V Leiden mutation and low S protein levels. At the time of presentation, no other symptoms were reported. Histopathology of both lesions revealed the presence of widespread intravascular thrombi. The therapeutic response to oral anticoagulants in the first case was dramatic, leading to complete healing of the lesions. Inherited causes of thrombophilia manifested as cutaneous lesions, although rare, should be considered in the differential diagnosis of occlusive vasculopathy. (C) 2004 Wiley-Liss, Inc