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Porphyria Cutanea Tarda

2024-03-06 11:22:27

Introduction: Porphyria cutanea tarda is described as photosensitive skin symptoms 2, 3. Along with this illness, a large amount of urinary excretion of porphyrin 2 appears in urine. There are two types of porphyria, sporadic and familial. Loose hair is originally classified by high levels of urinary porphyrin in the liver, but the family type has a 50% deficiency of precise enzymes. Porphyria is an autosomal dominant disease that occurs in adults and children.

Non-acute porphyria is X-linked dominant protoporphyrinopathy (XLDPP), congenital red blood cell producing porphyria (CEP), late porphyria (PCT) and erythroid protoporphyrin (EPP). ) These are not related to acute attacks, their main symptoms are skin diseases. For this reason, these four porphyria, and two acute porphyria, VP and HCP that may also be involved in skin symptoms are sometimes called skin porphyria. Skin disease accumulating too much porphyrin on the skin. Porphyrin is a photoactive molecule that raises electrons to higher energy levels when exposed to light. When they return to the resting energy level or ground state, the energy is released. This explains the typical fluorescence characteristics of porphyrin. This can cause local skin damage

Variegate porphyria caused by a partial deficiency of PROTO oxidase (aka Porphyria variegata or mixed porphyria) is a skin lesion resembling a porphyriosis skin disease with an acute nervous system attack. All other porphyria is primarily skin or nerve. Porphyria is diagnosed by biochemical analysis of blood, urine, feces. In general, urinary choline (PBG) urine is presumed to be the first step if you suspect acute porphyria. As a result of feedback, a reduction in hemoglobin results in an increase in the production of precursors and PBG is one of the first substances in the porphyrin synthesis pathway. In almost all cases of acute porphyria syndrome, urinary PBG is significantly elevated in patients with symptoms due to very rare ALA dehydratase deficiency or type I hereditary tyrosinemia.