A Case of Incontinentia Pigmenti

Son, Il Yeong; Yeo, Un Cheol; Lee, Eil Soo

Abstract

Incontinentia pigmenti is an uncommon neurocutaneous genodermatosis characterized by three stages; vesicular lesions, verrucous lesions and hyperpigmentation. A two-week-old female infant showed grouped erythema-based vesiculopustules on the whole body since birth. One month later, vesiculopustular lesions began to disappear gradually. At this time, she developed linear verrucous plaques on the dorsum of the feet and hands and reticulated hyperpigmented patches on the lower extremities. At 2 months of age, vesicular lesions completely disappeared and the pigmented patches spread to the abdomen which became darker with time. The verrucous lesions diminished at 3 months of age. There were no neurological or ocular defects. We describe a case of incontinentia pigmenti with typical clinical and pathological features at each stage.