Posterior reversible encephalopathy in a child with Langerhans cell histiocytosis following allogeneic PBSCT treatment with cyclosporine.

Posterior reversible encephalopathy syndrome (PRES) is associated with a specific disorder of cerebrovascular autoregulation of multiple etiologies. This syndrome had been subsequently described in numerous medical conditions, including hypertensive encephalopathy, pre-eclampsia and the use with immunosuppressive drugs. Here, we report a child suffering from Langerhans cell histocytosis developing into PRES following immunosuppressive therapy. Symptoms and neuroimaging abnormalities were complete resolution subsequent to the withdrawal of cyclosporine. Although PRES is rarely seen among children, it should always be considered in the differential diagnosis of acute neurological illness, especially undergoing immunosuppressive therapy.

Monozygotic twins concordant for idiopathic thrombocytopenic purpura and discordant for systemic lupus erythematosus and lupus nephritis.

We report on identical twin sisters with systemic lupus erythematosus and lupus nephritis after initial presentation as idiopathic thrombocytopenic purpura. The patients had diverse clinical signs, symptoms and pathological findings of lupus nephritis. The changes in antinuclear antibodies and anti-double-stranded DNA antibodies were quite different. We conclude that even with an identical genetic background and the same environment, the expression of systemic lupus erythematosus and subsequent progression to lupus nephritis in twins was distinct, and antinuclear antibodies and anti-double-stranded DNA antibodies are helpful for clinical monitoring.