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"What is porphyria cutanea tarda?  The porphyrias are a group of diseases in which there is a defect somewhere in the complex chain of chemical steps that are required for the synthesis of the molecule known as haem.  Haem is one of a group of compounds called porphyrins and is an iron-containing molecule that combines with the protein globin to form haemoglobin.  Haemoglobin is present within the red cells of the blood and is responsible for combining with and transporting oxygen around the body. The two main sites in the body for haem synthesis are the bone marrow and the liver.  In porphyria cutanea tarda (PCT) this enzyme defect may be within the liver and, in some people, also in the red blood cells.  The lack of this enzyme causes a build-up of porphyrins in the blood and skin. These react with light, making the skin fragile and easily blistered.  Some people are born with the condition, but in the majority it appears later in life, having been caused by a toxin (such as some pesticides and fungicides) affecting the liver.  Other contributing factors can be the intake of alcohol, iron supplements and oestrogen-containing medicines.  Which skin problems does it cause?  The skin, particularly on the backs of the hands and sometimes the face, becomes easily bruised and susceptible to fluid or blood-filled blisters. Scarring and small white skin cysts may develop. Occasionally, the affected skin becomes thick and hard.  The parts of the skin that are affected are those that are normally exposed to light. While occasionally sufferers notice that exposure to light causes their blisters, many others don&amp;#039;t realise that light is the source of the problem. This is primarily because it is the visible wavelengths rather than the sunburn-causing ultraviolet light that are responsible. Because visible wavelengths are always present, even in the winter months, this is potentially a year-round condition.  How is it diagnosed?  Sometimes it can be diagnosed by examining the affected person&amp;#039;s skin. In other people it&amp;#039;s more difficult, requiring blood, urine and stool samples to confirm the diagnosis \u2013 they will show elevated levels of porphyrins.  Other important tests may involve checking liver function, the iron levels in the blood, looking for previous liver viral infections, and taking a history to detect aggravating drugs.  Is it serious?  This is usually a nuisance rather than a serious condition. It is not associated with the development of skin cancer. However, sufferers have an increased risk of developing liver cancer (hepatocellular carcinoma), particularly if:   they are males.  over 50 years old.  have had symptoms of PCT for more than 10 years.  a history of excessive alcohol consumption or cirrhosis of the liver.   Can I pass it on to my children?  PCT, which is non-infectious, can on occasions be of a type that is inherited. This matter is best discussed with your doctor. It is true, however, that for most people the possibility of passing it on to their offspring is low.  Is any treatment available?  As PCT is always associated with excessive amounts of iron stores within the liver, one method of treatment that can be very effective is the regular withdrawal of blood, in a way similar to giving a blood donation. This technique, called venesection, reduces the body&amp;#039;s iron stores but the procedure does need to be repeated and also monitored carefully to ensure that enough iron stores are left for the body&amp;#039;s needs.  Chemicals or medicines that are toxic to the liver or are known to be capable of aggravating the condition, including iron supplements, should be avoided. Alcohol intake should be reduced or, preferably, stopped completely. Anyone with PCT should not take any medicine without discussing it with their doctor.  When the simple avoidance of liver-toxic chemicals is not enough, the medicines chloroquine (eg Avloclor, Nivaquine) \u2013 usually used in the treatment of malaria, or hydroxychloroquine (Plaquenil) may help by reducing liver porphyrin stores.  Avoiding sunlight  Because it can take several months for venesection or tablet treatment to help, avoiding light by wearing a broad-brimmed hat, thickly woven clothing and gloves when outdoors can be helpful. Also, there are sunscreens available that can help to reduce visible light acting on the skin. These can be used on particularly bright days.  As physical damage to the skin can lead to blistering and scarring, it is advisable to wear strong gloves when working.  Does the condition ever get better on its own?  For those who have alcohol-induced PCT, or if the condition is due to a drug, simply avoiding these can produce a return to normal skin. More often, however, the problem persists and needs intermittent treatment.  Other people also read: Cirrhosis of the liver: What are the symptoms of liver cirrhosis? Malaria: How do you catch malaria?  Based on a text by Professor James Ferguson, consultant dermatologist  Last updated 09.07.2014 "^^<http://www.w3.org/2001/XMLSchema#string>
<http://www.netdoctor.co.uk/conditions/skin-and-hair/a3634/porphyria-cutanea-tarda/>