Progression and Possible Complications of Porphyria

What is porphyria? (Header 1)

Porphyria is a group of rare disorders that affect the skin (cutaneous porphyria) and nervous system (acute porphyria). It involves a buildup of natural chemicals called porphyrins in the body. Porphyrins are necessary for the production of heme, which is a part of hemoglobin. Hemoglobin carries oxygen to organs and tissues.

In order to convert porphyrins into heme, eight enzymes are needed. Without these enzymes, porphyrins build up in the body. This results in major issues, especially regarding the nervous system and skin.

Progression

Symptoms of porphyria may be experienced throughout life, but often leave and reappear. Depending on the triggers, most people recover from symptoms.

Complications

The complications of porphyria are dependent upon the type. Acute porphyria has a rapid onset and presents mainly in the nervous system. Cutaneous porphyria has a slower start and impacts the skin.

Acute porphyria

If not treated quickly and properly, attacks of acute porphyria may be dangerous, or even life-threatening. Hospitalization is frequently necessary for treatment. Various complications may occur, including, but not limited to, the following:

• Dehydration
• Seizures
• Breathing problems
• High blood pressure

If multiple bouts of acute porphyria occur, long-term complications can develop. This may include the following:

• Chronic pain
• Liver damage
• Liver cancer
• Chronic kidney failure

Cutaneous porphyria

Cutaneous porphyria may result in permanent skin damage. Other potential complications include the following:

• Skin infections
• Discoloration of skin
• Skin scarring
• Increased risk of liver damage or liver cancer