Abstract: The clinical findings and experimental results of incontinentia pigmenti(IP) were analyzed.Of the 38 cases,37 are female and only one male,15 of them had skin rash at birth;22 of them had rash within 6 weeks after birth;One of them developed rash at the age of 9 weeks.16 of the patients had family history of IP.36 of them passed through three clinical stages:erythema-vesicular,verrucous plaque and pigmentation.2 of them had characteristic pigmentary maculae at birth.25 of the cases had extracutaneohs abnormalities,including eyes,teeth and central nervous system defects.During the erythema-vesicular stage of IP,the eosinophil count in 7 cases was predominantly increased.6 cases had had convulsion without fever and 3 of them showed hyperarrhythmia in EEG.Chromosome analysis was performed in the male case,and it showed 47,xxy karyotype.Skin biopsy of 7 cases showed typical pathological changes of IP.Although there is no effective prenatal diagnosis and special treatment for IP,establishment of genetic counselling service is important for the families with IP to improve birth quality.Prevention of secondary infection during erythema-vesicular stage and symptomatic treatment have to be given to the patients.

Key words: Incontinentia pigmenti