Incontinentia pigmenti (IP), also known as Bloch- Sulzberger’s disease, is a rare X-linked dominant genodermatosis caused by a mutation in nuclear factor- kB (NF- kB) essential modulator (NEMO) gene on the X chromosome, localized to Xq28. IP predominantly affects female infants (in excess of 37 : 1), and is usually lethal in males in utero. The survival of affected males is attributed to the presence of an extra X chromosome (Klinefelter’s syndrome), hypomorphic mutations, and somatic mosaicism. Only two cases of IP in male infants without karyotype study have been reported in the Korean dermatologic literature since 1982 . This study reports a rare case of incontinentia pigmenti in normal karyotype (46, XY) male infant.
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