FIGURE 67.5 Linear arrangement of lesions (blisters in some cases) in an infant with incontinentia pigmenti Incontinentia Pigmenti Incontinentia pigmenti, a rare condition, occurs almost exclusively in females as it is inherited in an X-linked dominant fashion Inflammatory vesicles and bullae erupt in crops in a linear or curvilinear distribution (especially on the extremities) for the first several weeks to months of life ( Fig 67.5 ) These affected areas then progress to a warty stage Finally, swirl-like pigmentation occurs but not necessarily in the areas previously involved with warty or blistering lesions During the vesiculobullous stage, peripheral eosinophilia occurs (18% to 50% eosinophils) These patients often have extracutaneous manifestations, including dental anomalies, neurologic issues, ophthalmologic disease, and developmental