Q80.4: Harlequin fetus

You have a severe congenital skin disorder where the skin cells do not keratinize properly.

The top of layer of skin is made up of skin cells that are keratinized. These skin cells are initially in one of the lower skin layers. When the skin cells migrate upwards toward the skin’s surface, they keratinize, forming a solid layer.

The genetic information in your cells has changed. This disrupts the keratinization of your skin.

The condition causes the skin to form a firm layer even before birth. This firm layer encloses the body like a shell. The firm layer can limit mobility and therefore also impede breathing. You may then be very severely ill. It is also possible for the firm layer to result in malformations. Things like ears, eyelids, lips or fingers may be malformed.

Later, the skin may be red and scaly. You can get colder than usual or lose fluid through the skin. It is also possible for the skin to become inflamed more easily than usual or for large wounds to form.

This disease is hereditary. Other family members may also be affected.

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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