Depigmented Lesions in Incontinentia Pigmenti

Abstract

Depigmented lesions of the skin are not commonly appreciated as manifestations of incontinentia pigmenti. Their incidence and pathogenesis are unknown; their histologic characteristics are poorly defined. They usually form streaked, hypomelanotic macules in the calves but may be found in other areas. Their presence in adult relatives of children with incontinentia pigmenti may provide the only clue to the inherited character of the disease in a specific family. Thus, a search for these lesions plays an important role in the genetic counseling of these families.