Incontinentia pigmenti is caused by a mutation in NEMO/IKKγ gene which locates on X chromosome, which is lethal for male fetuses. It involves skin, teeth, hair or neurologic system. The cutaneous lesions distribute on the extremities along Blaschko’s lines, which usually occur in sequelae including vesicular, verrucous, hyperpigmented and atrophic stages. The vesicles usually are found at birth or within a few weeks of life and resolve spontaneously in a few days to weeks. The diagnosis is relatively easy if the the baby present with typical skin manifestations. The data of a female relative with confirmed incontinentia pigmenti or a gene defect of NEMO has a significant role for the diagnosis. Here we report a case of incontinentia pigmenti in a neonate girl with blisters.
Keywords
- Incontinentia pigmenti
- NEMO
- Inhibitor kappa kinase
- IKK