Incontinentia Pigmenti

**Semantic type:** Disease or Syndrome

**Definition:** A rare disorder caused by mutations in the IKBKG gene. It is characterized by skin abnormalities, alopecia, dystrophic nails, and abnormal tooth shape. The skin changes evolve from an initial blistering rash, to wart-like lesions, and eventually to hypopigmentation.

**Synonyms:** - Incontinentia Pigmenti Syndrome