Q82.3: Incontinentia pigmenti

You have a congenital disorder that causes changes in various parts of your body.

The disorder is hereditary. Other family members may also be affected.

The disorder usually causes lesions on the skin that are shaped like stripes. The skin frequently has a blistering rash at birth and is reddened in the affected areas. Later, these areas may begin to look like warts, flake off and turn dark.

The disorder can also affect other areas of the body. The eyes or teeth may not develop properly. The nervous system can also be affected. The nervous system includes all the nerves in the body, as well as the brain and the spinal cord. The nerves run from the brain and spinal cord into the body. They convey information in the body. If the nervous system is affected, you may have seizures or be unable to move normally, for example. You may be seriously ill.

Additional indicator

On medical documents, the ICD code is often appended by letters that indicate the diagnostic certainty or the affected side of the body.

  • G: Confirmed diagnosis
  • V: Tentative diagnosis
  • Z: Condition after
  • A: Excluded diagnosis
  • L: Left
  • R: Right
  • B: Both sides

Further information


This information is not intended for self-diagnosis and does not replace professional medical advice from a doctor. If you find an ICD code on a personal medical document, please also note the additional indicator used for diagnostic confidence.
Your doctor will assist you with any health-related questions and explain the ICD diagnosis code to you in a direct consultation if necessary.


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