Recurrent inflammation in incontinentia pigmenti of a seven-year-old child.

A young girl has undergone the typical skin manifestations of incontinentia pigmenti, leaving now only discrete hyperpigmentation on the flexor sides of the lower extremities. Each time when she has a feverish infection, a recurrence of inflammation can be seen within the hyperpigmented areas. At the same time when the fever subsides, the acute skin manifestations disappear as well. The combination of recurrent inflammatory skin manifestations within the hyperpigmented skin areas of incontinentia pigmenti and fever is unique. The reasons for this phenomenon and for its strict limitation to the areas of incontinentia pigmenti remain unclear.