Porfyri akut cutanea tarda
Hem / Hälsa & Välmående / Porfyri akut cutanea tarda
Exposure to sunlight activates the accumulated porphyrins in the skin and results in blistering. Nevertheless, it seems worth first treating and curing hepatitis C virus infection with direct antiviral treatment before deciding on iron reduction or hydroxychloroquine therapy in such patients (). Also, liver damage progresses slowly if at all in many people with HCV.
However, once the PCT is in remission it is important to assess the amount of liver damage the virus has already caused and to have follow-up visits to a doctor to monitor the liver. Iron chelators are less effective than phlebotomy or low dose hydroxychloroquine or chloroquine in treating individuals with PCT. However, these drugs may play a role in treating affected individuals in whom the use of the two front-line therapies is not possible, such as individuals with end stage renal disease who are on hemodialysis.
Affected individuals are advised to avoid environmental triggering factors of the disorder such as alcohol and smoking.
Some people with PCT can develop cirrhosis (chronic liver damage and scarring) and even liver cancer.
Only the familial form of PCT can be inherited, and even in this form people with PCT generally do not have any family members with symptoms. This uses up excess iron by making new red blood cells.
What is the outlook for porphyria cutanea tarda?
Once clear, PCT is unlikely to recur unless the underlying risk factors have not been addressed.
Ongoing iron reduction therapy is also recommended when hereditary hemochromatosis is present. Commonly implicated medications are furosemide, tetracyclines, sulfonamides, and naproxen and other nonsteroidal anti-inflammatory drugs (NSAIDs). Depending on the degree of hemodilution and iron depletion, the skin symptoms usually abate as pregnancy advances.Skin symptoms occurring during pregnancy are treated with phlebotomy.
Treat patients with HIV infection with antiretroviral therapy.
More Information
The following English-language resources may be useful. Individuals with PCT present with increasingly fragile skin on the back of the hands and the forearms. Dialyzers with ultrapermeable membranes and extra high blood flow rates are needed.
Patients with overt PCT and hepatitis C infection should be evaluated for treatment with direct-acting antiviral drugs.
Some bullae are hemorrhagic.
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Spontaneously or after minor trauma, tense bullae develop. HEP occurs in childhood and is usually more severe than PCT types 1 or 2. Affected individuals are particularly susceptible to damage of the skin from sunlight (photosensitivity).
Eventually, scarring may develop, and affected skin may darken (hyperpigmentation) or fade (hypopigmentation) in color. PCT caused by hepatitis C can be treated with one of the antiviral regimens to remove that specific risk factor.
The risk factors for developing PCT symptoms should be avoided while someone is having symptoms.
Commonly implicated medications are furosemide, tetracyclines, sulfonamides, and naproxen and other nonsteroidal anti-inflammatory drugs (NSAIDs).
Because porphyrins are poorly dialyzed, some patients receiving long-term hemodialysis develop a skin condition that resembles PCT; this condition is termed pseudoporphyria of end-stage renal disease.
Some bullae are hemorrhagic. Typical sunscreens that block UV light are ineffective, but UVA-absorbing sunscreens, such as those containing dibenzylmethanes, may help somewhat. This mutation is inherited as an autosomal dominant trait. It usually manifests in middle age or later.