An instance of incontinentia pigmenti achromians1 occurring in a 3-month-old Japanese girl is reported. Since the age of 1 month, sharply demarcated, linear or band-like depigmented macules of bizarre irregularity gradually developed on the trunk and extremities. Patterns of the depigmented lesions were similar to a negative picture of pigmented macules in patients with incontinentia pigmenti2,3. The association of internal strabismus, tessellated fundus, diffuse alopecia, and luxatio coxae congenita was also found. Microscopic studies of a depigmented area showed little or no inflammatory changes, but a decreased amount of melanin granules at the basal layer, where the epidermal melanocytes were weaker in dopa reaction. At the age of 16 months, lesions became obscure on the torso, but remained on extremities. Whether this hypomelanotic disorder is a true entity or variant of depigmented nevi is discussed.
Hamada T, Saito T, Sugai T, Morita Y. "Incontinentia Pigmenti Achromians (Ito)". Arch Dermatol. 1967;96(6):673-676. doi:10.1001/archderm.1967.01610060067009