Incontinentia pigmenti achromians (Ito).

A case of incontinentia pigmenti achromians syndrome with associated abnormalities occurring in a 21-year-old woman is reported. Since birth she has had whorling macular, hypopigmented areas on the skin of the lateral, anterior, and posterior aspects of the trunk. Histopathological picture: the melanocytes in the depigmented areas are slightly reduced in number. Some of the associated abnormalities that she exhibits, such as coarse curly hair on her scalp, and pigmented spots in her left iris, have never before been described. Other of her symptoms--thick lips and myopia--have been reported earlier. The patient's mother, 15-year-old sister and 9-year-old brother showed, from birth, brown hyperpigmented maculae on the left halves of their bodies.