Parents

IP manifests in four skin stages that evolve sequentially: the bullous stage, the verroucous stage, the hyperpigmentation stage, and the hypopigmentation stage. The onset and duration of each stage vary among individuals, and not all individuals experience all four stages. The skin abnormalities that define each stage occur along lines of embryonic and fetal skin development known as Blaschko’s lines. Many other tissues can be affected in IP, including hair, teeth, eyes, and CNS. More than 2,000 females with IP have been reported. The number of reported females and males continues to grow, especially with further delineation of the underlying molecular mechanisms. Orphanet recently estimated prevalence at birth of 1.2/100,000 in the European Union.