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Abstract [Objective]To explore clinical features of incontinentia pigmenti(IP)in children in order to im-prove the awareness of the disease.[Methods]Clinical features of 1 1 pediatric patients with IP in our hospital from Jan.2004 to Dec.2013 were analyzed.[Results]Among 11 pediatric patients,10 patients were female and 1 patient was male.Two patients were premature twins.Eleven patients had skin involvement.Two pa-tients had nerve system involvement and convulsion.Two patients had eye involvement.The percentage of eo-sinophils in peripheral blood of 6 patients was obviously increased.Skin pathological biopsy of 4 patients showed a large of eosinophils in vesication to confirm the diagnosis.Gene deletion of nuclear factor kB essential regulator(NEMO)was detected in 1 patient.[Conclusion]IP is a rare x-linked dominant genetic disease.The dominant feature in neonatal period is skin lesion.It can be involve in multiple systems.Eye and nervous sys-tem lesions are serious.Skin pathology and chromosomal genetic analysis are the methods of definite diagno-sis.Pediatric patients should be followed up regularly.
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2014, Vol. 31 
