Incontinentia pigmenti: clinicopathologic characteristics and differential diagnosis

A newborn girl had erythematous-based vesicles and pustules on her chest and extremities and eosinophilia. Within two weeks, she also showed linear verrucous plaques on her arms and legs. Pathologic evaluation of the skin lesions confirmed the clinically suspected diagnosis of incontinentia pigmenti. Results of subsequent neurologic, ophthalmologic, and pediatric evaluations have been normal without the detection of potential incontinentia pigmenti-associated systemic manifestations. The clinical and pathologic features of incontinentia pigmenti skin lesions are reviewed and the systemic manifestations summarized.