Conclusion: We recommend a uniform parlance when describing the clinical picture of pigmentary mosaicism.

We have compiled information on previous cases of pigmentary mosaicism aiming to optimize the handling of patients with this condition.

In a substantial number of cases, pigmentary mosaicism is observed alongside extracutaneous abnormalities typically involving the central nervous system and the musculoskeletal system.

Background: Pigmentary mosaicism is a term that describes varied patterns of pigmentation in the skin caused by genetic heterogeneity of the skin cells.