In the long list of dermatological conditions is an inborn metabolic disorder by the name Porphyria Cutanea Tarda (PCT). Considering how rare the skin condition is, we won’t blame you if you have never heard of it before! Nevertheless, it’s important to understand what causes it and how you can seek treatment if you or someone you know is suffering from it.
What Is Porphyria Cutanea Tarda?
A group of diseases that occur due to the build-up of natural chemicals that produce porphyrin (a photosensitive pigment that is responsible for delivering oxygen to body tissues) in your body is called porphyria. There are two general types of porphyria
- Acute: The acute variety of porphyria affects the nervous system.
- Cutaneous: The cutaneous variety of porphyria affects the skin.
Certain porphyria types display both of the above variations. PCT is a type of porphyria, or blood disorder which manifests itself in the form of erosions, bullae, milia, and scars. This manifestation is caused when excess porphyrins in the skin interact with light. This is why PCT is most likely to occur on sun damaged or sensitive skin.
PCT can be categorized into three types – familial, acquired or toxic. Factors inducing PCT include alcohol, estrogen, iron, polyhalogenated compounds (like DDT and pesticides) and viral infections.1
What Are The Symptoms Of PCT?
The most common symptoms of PCT include
- Blisters or bullae
- Milia (small white bumps)
- Increased skin fragility
- Hyperpigmentation (dark skin patches)
- Hypertrichosis (excessive hair growth)
- High levels of porphyrin in urine
- Accumulation of calcium in degenerated tissues
Other disorders associated with this skin condition include diabetes mellitus, systemic lupus erythematosus (the most common form of lupus, an autoimmune disease), elevated serum iron levels, and abnormal liver function.2
Forms Of PCT
The two observed forms of PCT are familial (or genetic) and acquired. Here are the differences between them
- Genetic PCT: In the generic variety of this skin condition, patients develop symptoms due to hepatic (liver) or erythrocyte (red blood cells) deficiency of uroporphyrinogen decarboxylase (also called UD). UD is an enzyme involved in the production of heme molecules, which are found in hemoglobin.
- Acquired PCT: Patients with this form of PCT develop symptoms when exposed to hexachlorobenzene (HCB) (a carcinogen), a fungicide, or polychlorinated phenols (a pesticide).3
Alcohol, estrogen, iron overload, and hepatitis C virus infection are known to aggravate PCT. Studies also suggest that there is an association between HIV infection and PCT.4
Treatment Options For PCT
There are several treatment options for PCT.5 These include
- Phlebotomy: Phlebotomy or removal of blood, is often used to treat PCT. This is especially helpful for those with a high iron burden. However, care needs to be taken so as to ensure that the it doesn’t lead to anemia. Although relapses do occur, they can be treated with incremental schedules of phlebotomy. At the moment, this line of treatment is the most successful.
- Oral chloroquine: Low doses of oral chloroquine is another option for PCT patients. However, there is a chance of relapse with this treatment as well. Sometimes, it could even lead to an adverse toxic reaction. But, considering the fact that the side effects are not permanently damaging, this treatment is still recommended by professionals.
- Hydrochloroquine therapy: This option is believed to be safe and effective in the treatment PCT because it helps normalize the urinary excretion of porphyrins. Do consult a medical professional to see how feasible this option is for you.
If all the symptoms mentioned above are a regular occurrence for you, then it’s important for you to seek medical help immediately so as to rule out the possibility of PCT . If you have sensitive skin or are prone to PCT, then you should follow a doctor-approved skin care regimen. Additionally, you might also need to avoid sunlight as much as possible. Apart from this, it’s also important to avoid triggers like alcohol and tobacco.
Since PCT is rare, being diagnosed with it might be overwhelming and confusing at first. But, a few lifestyle changes coupled with a little care can help patients with this skin disorder manage and beat the disease with ease.
|↑1||Bleasel, Narelle R., and George A. Varigos. “Porphyria cutanea tarda.” Australasian Journal of dermatology 41, no. 4 (2000): 197-208.|
|↑2, ↑5||Grossman, Marc E., David R. Bickers, Maureen B. Poh-Fitzpatrick, Vincent A. Deleo, and Leonard C. Harber. “Porphyria cutanea tarda: clinical features and laboratory findings in 40 patients.” The American journal of medicine 67, no. 2 (1979): 277-286.|
|↑3||MUHLBAUER, JAN E., and MADHU A. PATHAK. “Porphyria cutanea tarda.” International journal of dermatology 18, no. 10 (1979): 767-780.|
|↑4||Fritsch, Clemens, K. Lang, S. Von Schmiedeberg, K. Bolsen, H. Merk, P. Lehmann, and T. Ruzicka. “Porphyria cutanea tarda.” Skin Pharmacology and Physiology 11, no. 6 (1998): 321-335.|