Clinical Study of Incontinentia Pigmenti in 10 Cases of Neonates

Objective To investigate the clinical manifestations of incontinentia pigmenti(IP) in the neonatal period,as well as the diagnosis and prognosis. Methods The clinical manifestations and prognosis of 10 neonatal IP patients,hospitalized at Beijing Children′s Hospital from Oct.2002 to Mar.2011,were analyzed.All the patients were diagnosed according to Landy-Donnai criteria.All the patients were full-term baby girl,with median birth weight 3 350 g.There were 4 cases out of 10 patients survived with median following 43 months,till Jul.1.2012,except 4 cases died in the following period and 2 cases lost follow-up.The clinical manifestations including skin changes,eye abnormalities and nervous system damage,and assistant examinations,as well as their contributions to diagnosis and prognosis were analyzed.SPSS 16.0 software was used in this study for statistical analysis. Results Performance of 10 patients were skin rash,including erythema,papules,blisters,verrucous hyperplasia and ink-like pigmentation.Eosinophils in peripheral blood significantly increased in 6 patients.Two patients existed eye abnormalities during hospitalization.Four patients experienced convulsions in neonatal period,with poor anticonvulsant therapy effect and 3 cases died.One patient experienced convulsions after neonatal period,in the follow-up,with good anticonvulsant response.Further,whether experienced convulsions in neonatal period were related to the survival rate(P=0.002 4). Conclusions Skin rash was the main change in neonatal patients suffered IP.Those patients who accompanied by nervous system damage especially had a poor prognosis.