<unk>  <unk>  is recognized as the most prevalent subtype of<unk> ic diseases.<unk>  is characterized by onycholysis and blistering of the skin in areas that receive higher levels of exposure to sunlight. The primary cause is a deficiency of uroporphyrinogen decarboxylase (UROD), a cytosolic enzyme that is a step in the enzymatic pathway that leads to the synthesis of heme. Behind the direct cause there are a number of genetic and environmental risk factors. Patients who are diagnosed with<unk>  typically seek treatment following the development of photosensitivities causing blisters and erosions on exposed areas of the skin. This is usually observed in the face, hands, forearms, and lower legs. Healing is slow and leaves scarring. Though blisters are the most common skin manifestations of<unk> , other skin manifestations include hyperpigmentation (similar to a tan) and hypertrichosis (mainly on the cheeks) also occur.<unk>  is a chronic condition, with external symptoms often subsiding and recurring as a result of multiple factors. In addition to the skin lesions, chronic liver disease is very common in patients with sporadic<unk> . This involves hepatic fibrosis (scarring of the liver), and inflammation. However, liver problems are less common in patients with the inherited form of the disease. Additionally, patients will often void a wine-red color urine with an increased concentration of uroporphyrin I due to their enzymatic deficiency. Vitamin, mineral, and enzyme deficiencies Certain vitamin and minerals deficiencies are common in people with<unk> . The most common deficiencies are beta-Carotene, retinol, vitamin A and vitamin C. Beta-Carotene is required to synthesize vitamin A and vitamin A is needed to synthesize retinol. A lack of retinol-binding protein is due to a lack of retinol which is required to trigger its production.<unk> s interact with iron, absorbing photons to create reactive oxygen species is the mechanism of action causing the itchy, painful blisters of<unk> . The reactive oxygen species consume the skin antioxidants beta-carotene, vitamin E, and vitamin C. Supplementation of these three vitamins reduces the oxidation and potentially diminishes the severity of blister formation. No single one of the three vitamins can inhibit the damaging effects of oxidized<unk> s, specifically uroporphyrins and coproporphyrins, but all three working together synergistically are capable of neutralizing their damaging effects.
Porphyria cutanea tarda
Prurigo pigmentosa
Phakomatosis pigmentovascularis
Dermatopathia pigmentosa reticularis
Urticaria pigmentosa
Variegate porphyria
Hepatic porphyria
Phakomatosis pigmentokeratotica