Incontinentia pigmenti achromians (Ito)

A patient with incontinentia pigmenti achromians was reported. She displayed other abnormalities such as a saddle nose, malformed auricles, growth retardation, convulsions and abnormal EEG. Histopathologically, a partial decrease in melanin granules in the basal layer and the presence of melanocytes which showed a weak dopa reaction were the main findings. The hypofunction of melanin production was detected by electron microscopy.