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Health Inset > Diseases and Conditions > Porphyria cutanea tarda

Porphyria cutanea tarda

What is Porphyria Cutanea Tarda?

Porphyria cutanea tarda (PCT) is a type of porphyria (genetic metabolic abnormality) that arises most frequently. The disease is caused by a deficiency in the levels of the enzyme known as uroporphyrinogen decarboxylase (UROD) which is responsible for the 5th step in the production of heme, a hemoglobin component. PCT makes the skin of its patients photosensitive.

Porphyria Cutanea Tarda Epidemiology

The disease is known to affect 1 in every 10000 individuals.

Porphyria Cutanea Tarda Types

There are two types of PCT: sporadic and familial. A third type is also included by some sources, although this is less prevalent.

Type I

It is the sporadic form. It is not inherited and is the most common form of the disorder (80%).

Type II

It is the inherited or ‘familial’ variant of the condition and affects nearly 20% of patients. Although a genetic factor is responsible for the development of Type II PCT, many people might spend their entire lives without any visible sign of the disorder.

Type III

This is the least common form of PCT. Although individuals affected by this condition have normal UROD genes like in Type I, a genetic factor unrelated to UROD expression is believed to be the underlying cause behind this condition.

Porphyria Cutanea Tarda Causes

PCT results from a deficiency of the cytosolic enzyme called uroporphyrinogen decarboxylase that is involved in an enzymatic pathway in heme synthesis. Although the enzyme deficiency is stated to be the primary factor responsible for this disorder, it is an acquired condition in most cases. The various risk factors that can accentuate or trigger PCT include the following:

  • Fasting
  • Smoking
  • Infections
  • Chemicals
  • Menstrual hormones
  • Exposure to sun’s rays
  • Exposure to Agent Orange
  • Physical and emotional stress
  • Human immunodeficiency virus (HIV)
  • Viral infections of liver, such as hepatitis C
  • Exposure to various chlorinated cyclic hydrocarbons
  • Consumption of alcohol, and habits of heavy drinking
  • Drugs such as tranquilizers, barbiturates, sedatives and birth control pills
  • Excess amounts of iron in the body, or the inherited condition of hemochromatosis
  • Estrogen therapy, such as oral contraceptives and/or or hormone replacement therapy (HRT)

The condition can also be paraneoplastic.

Porphyria Cutanea Tarda Symptoms

The main symptoms exhibited by PCT patients include the development of blisters and erosions due to photosensitivity reactions on the commonly exposed surfaces of the skin. These are mostly observed on the face, forearms, hands, and lower legs. The skin might become hardened (sclerodermoid) around the face, neck or chest. There might be signs of ulcers or small portions affected by alopecia (permanent baldness). Other skin manifestations may comprise of hyperpigmentation and hypertrichosis.

Apart from the skin symptoms mentioned above, sufferers might also exhibit chronic liver problems which are quite commonly seen in patients having sporadic form of Porphyria Cutanea Tarda. These include scarring of liver or hepatic fibrosis, cirrhosis, and/or inflammations. However, the patients suffering from the inherited variant of the disease are much less likely to develop the liver ailments.

The urine might exhibit a coloration that is darker than normal.

Porphyria Cutanea Tarda Diagnosis

Although the most common symptoms and signs of PCT include appearance of blisters and skin lesions, their presence do not conclusively prove the diagnosis of this condition. Lab tests will generally reveal presence of high quantities of uroporphyrinogen in one’s urine; a condition commonly termed as uroporphyrinogenuria. Tests also should be conducted for the usual risk factors like hemochromatosis and Hepatitis C infections, as high prevalence of these factors in PCT patients might require special treatment. A diagnosis of pseudoporphyria needs to be considered seriously if the lab tests come out as negative but the condition exhibits all the clinical signs of porphyria cutanea tarda.

The diagnostic tests conducted to evaluate the presence of PCT include the following:

  • Skin biopsies help in differentiating this disorder from other similar skin conditions
  • 24-hour urine porphyrin profile generally reveals 5- to 20-fold excess porphryin levels as well as presence of uroporphyrin and 7-carboxyl porphyrin
  • Urine examination under a Wood’s lamp might reveal presence of coral pink fluorescence caused by excessive porphyrins

Other important diagnostic procedures include:

  • Complete blood count for assessing hemoglobin levels
  • Measuring total iron content
  • Liver enzyme tests
  • Stool tests
  • Viral hepatitis studies
  • DNA studies
  • Fasting blood sugar due to the increased frequency of diabetes
  • Antinuclear antibodies due to increased occurrence of lupus erythematosus

Porphyria Cutanea Tarda Differential Diagnosis

There are a number of other skin conditions such as photo-aggravated bullous dermatoses, porphyrias and pseudoporphyrias which might manifest clinical features similar to that of PCT. It is important to obtain adequate biochemical data to accurately diagnose porphyria cutanea tarda, without which the treatment measures may become faulty. The differential diagnoses for PCT include ensuring the absence of conditions such as:

  • Pseudoporphyria
  • Hydroa Vacciniforme
  • Epidermolysis Bullosa
  • Erythropoietic Porphyria
  • Bullous Lupus Erythematosus
  • Epidermolysis Bullosa Acquisita

Porphyria Cutanea Tarda Treatment

As PCT is a chronic disease, the best way to manage this condition is to follow it up diagnosis with a comprehensive treatment regime. Firstly, it is essential that the patient diagnosed with PCT avoids all factors which might potentially exacerbate this disorder. These include:

  • Consumption of alcohol
  • Intake of iron supplements
  • Excessive exposure to sunlight
  • Chlorinated cyclic hydrocarbons
  • Estrogen

The presence of excess amounts of iron can be managed by a process known as phlebotomy, in which blood is systematically removed or drained from the body. Autologous red cell transfusion helps in reducing the circulating porphyrins by washing the plasma away from the red blood cells of affected individuals. Low quantities of antimalarial medications can also be used. They help in removing excess porphyrins from liver by increasing rate of excretion. Signs of remission can become apparent within 6 to 12 months. Higher doses of these medications were once used to cure this condition, although at present it is not commendable to do so because of resultant liver toxicity.

Since there is a strong association between porphyria cutanea tarda and Hepatitis C, treatment of Hepatitis C (in case it is present), is essential to an effective management of PCT. Hydroxychloroquine, chloroquine, and venesection are generally used in PCT management. Patients are also advised to use tanning cream with dihydroxyacetone as well as an opaque sun-blocking cream.

Porphyria Cutanea Tarda Prognosis

The treatment for PCT is nearly always successful, with patients generally having an excellent prognosis. Treatment should be followed by periodic measurements of plasma porphyrins, especially in cases where a contributing factor like estrogen treatment is resumed. Although the condition might heal with treatment, it often leaves scars.

PCT is a chronic disorder, with the external signs and symptoms frequently subsiding and recurring due to several factors. In case there is a recurrence, it can be identified quickly and treated promptly.

Porphyria Cutanea Tarda Pictures

Here are some images that will give you a visual estimate of the dermatological symptoms associated with this condition.

Picture of Porphyria cutanea tarda

Picture 1 – Porphyria cutanea tarda

Image of Porphyria cutanea tarda

Picture 2 – Porphyria cutanea tarda Image

Of all the porphyric conditions, Porphyria cutanea tarda is the most treatable. The outcome of the treatment is very good, with patients leading healthy and normal lives after medical intervention.

References:

http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/PCT

http://www.dermnetnz.org/systemic/porphyria-cutanea-tarda.html

http://www.medicinenet.com/porphyria/article.htm

http://www.bad.org.uk/site/861/Default.aspx

http://rarediseasesnetwork.epi.usf.edu/porphyrias/patients/PCT/

By   Last updated February 22nd, 2013

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