Incontinentia pigmenti (aka Bloch-Sulzberger Syndrome) is skin condition passed down through families. It leads to unusual blistering and changes in skin color.

See also: Incontinentia pigmenti achromians

Infants with IP are born with streaky, blistering areas. When the areas heal, they turn into rough bumps. Eventually, these bumps go away, but leave behind darkened skin, called hyperpigmentation. After several years, the skin returns to normal. In some adults, there may be areas of lighter colored skin (hypopigmentation).

IP is associated with central nervous system problems, including:

• Delayed development
• Loss of movement (paralysis)
• Mental retardation
• Muscle spasms
• Seizures

Persons with IP may also have abnormal teeth, hair loss, and visual problems.

There is no specific treatment for IP. Treatment is aimed at the individual symptoms. For example, glasses may be needed to improve vision. Medicine may be prescribed to help control seizures or muscle spasms.

Incontinentia pigmenti (IP) is caused by a genetic defect. In most cases, there is a problem with one of the genes located on the X chromosome.

See also: X-linked trait

The condition is most often seen in females. When it occurs in males, it is deadly; it is extremely rare that a conceived male child is born alive as they are spontaneously aborted. 

Most babies born with IP develop discolored skin within the first 2 weeks. The discolored skin occurs when a substance called melanin builds up under the skin. Melanin gives skin its color.

A Wood's lamp examination of the skin lesions may help confirm the diagnosis. Your doctor may also recommend chromosome analysis or a further medical workup to discover any related medical problems

There may be unusual patterns and blisters on the skin, as well as bone abnormalities. An eye exam may reveal cataracts, strabismus  (crossed eyes), or other problems.

How well a person does depends on the severity of central nervous system involvement and eye problems.

Genetic counseling may be helpful for those with a family history of IP who are considering having children.

• Delayed development
• Infections of blistered skin
• Mental retardation
• Muscle spasticity
• Paralysis
• Seizures
• Walking difficulty
• Vision problems