E80.0: Hereditary erythropoietic porphyria

You are unable to produce a certain substance in the body properly because you have certain proteins that are not working properly. As a result, you are probably having problems with your skin.

The substance heme has various tasks in the body. For example, heme is contained in the red blood pigment. The red blood pigment transports oxygen in the blood vessels to the organs. Various proteins are needed to create heme. In the process, precursors of heme come into being. These precursors are called porphyrins.

In your case, a particular protein is not working properly. This condition is congenital, which means you were born with it. As a result, the heme in the bones especially cannot be properly created.

The precursors are then accumulating in your body. The precursors are predominantly deposited in the skin.

Your skin thus reacts very strongly to sunlight. Your skin is severely damaged by the sun. The skin then usually blisters and scars. Sometimes you may also get abdominal pain or circulation problems, for example. If the disease is very pronounced, you may be severely 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

Note

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.

Source

Provided by the non-profit organization “Was hab’ ich?” gemeinnützige GmbH on behalf of the Federal Ministry of Health (BMG).