What is Incontinentia Pigmenti

Incontinentia Pigmenti (IP) is a rare genetic disorder that primarily affects the skin, hair, teeth, and central nervous system. It predominantly affects females, with symptoms appearing shortly after birth. IP is caused by a mutation in the IKBKG gene, which is responsible for producing a protein involved in various cellular processes.

The characteristic features of IP include skin abnormalities, such as blistering, rashes, and pigmentation changes. These skin manifestations often follow a distinct pattern, starting as red, blister-like lesions that evolve into swirling patterns of hyperpigmentation. Hair abnormalities, such as patchy hair loss or unusually coarse hair, are also common.

IP can also affect the teeth, leading to delayed eruption, missing teeth, or abnormal tooth shape. Additionally, it may impact the central nervous system, causing developmental delays, intellectual disabilities, seizures, or vision problems.

While there is no cure for IP, treatment focuses on managing symptoms and preventing complications. Regular dermatological and dental care, along with early intervention programs, can help improve the quality of life for individuals with IP.