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A 24-year Korean woman presented with bizarre pigmentary skin changes.
Eruptive vellus hair cysts (EVHC) were observed in conjunction with Hypomelanosis of Ito (HI). Also, the karyotype of 45XO with typical clinical symptoms of Turner's syndrome was detected in this patient, which has been rarely reported in HI as a chromosomal defect. EVHC may manifest as a rare skin manifestation of Hl.