What does cutaneous porphyria look like?

What does cutaneous porphyria look like?

Sensitivity to the sun and sometimes artificial light, causing burning pain. Sudden painful skin redness (erythema) and swelling (edema. Blisters on exposed skin, usually the hands, arms and face. Fragile thin skin with changes in skin color (pigment)

What is a usual presentation of porphyria cutanea tarda?

The most common presenting sign of porphyria cutanea tarda is fragility of sun-exposed skin after mechanical trauma, leading to erosions and bullae, typically on hands and forearms and occasionally on face or feet.

How is porphyria cutanea tarda diagnosed?

To diagnose porphyria cutanea tarda, doctors test blood, urine, and stool for unusually high levels of porphyrins. The specific porphyrins that are increased provide a pattern that allows doctors to distinguish porphyria cutanea tarda from other porphyrias.

What triggers porphyria cutanea tarda?

Porphyria cutanea tarda (PCT) is usually acquired but may be hereditary. Triggers include elevated hepatic iron, alcohol use, halogenated hydrocarbon exposure, and hepatitis C virus or HIV infection. Drugs that commonly trigger acute porphyria do not trigger PCT.

How is cutaneous porphyria diagnosed?

The cutaneous porphyrias are all accompanied by elevated total plasma porphyrins, and are specifically diagnosed by measurements of porphyrins in red blood cells, plasma, urine, and stool, as well as by genetic or enzyme analysis.

What are the symptoms of porphyria cutanea tarda?

Common symptoms include:

  • blisters on skin that’s exposed to the sun, including the hands, face, and arms.
  • photosensitivity, which means your skin is sensitive to the sun.
  • thin or fragile skin.
  • increased hair growth, usually on the face.
  • crusting and scarring of the skin.
  • redness, swelling, or itching of the skin.

Is porphyria cutanea tarda a liver disease?

Porphyria cutanea tarda is a rare condition of the liver but it must be remembered in a differential diagnosis of liver disease with typical skin involvement to decrease morbidity and health costs with early treatment.

Is porphyria cutanea tarda serious?

General Discussion. Porphyria cutanea tarda (PCT) is a rare disorder characterized by painful, blistering skin lesions that develop on sun-exposed skin (photosensitivity). Affected skin is fragile and may peel or blister after minor trauma. Liver abnormalities may also occur.

Can porphyria cutanea tarda be cured?

PCT is the most treatable form of porphyria and treatment appears equally effective for both the sporadic and familial forms. The standard treatment of individuals with PCT is regularly scheduled phlebotomies to reduce iron and porphyrin levels in the liver.