Porphyria Cutanea Tarda or pseudoporphyria cutanea tarda

Dear Dr Agar,

I am just writing to you wondering if in your experiences over the years you have had a patient who developed PCT or PPCT.
due to long term dialysis.

My husband has developed this condition on his hands/face/ ears. It is a blistering condition that turns to sores and are prone to secondary infection which puts him in hospital for antibiotics.

He has been on dialysis for 10 years. previous before a transplant which lasted 17 years he was on dialysis for 5 years.

He also had cirrhosis of the liver (due to hepC) which I am told can also cause porphyria cutanea tarda. This skin condition is extremely bothersome and worrying. Sunlight or injury aggravates the condition and so he has to wear gloves/long sleeves/ hat while venturing outside for even a few minutes as this is all it takes. Some visual light also aggravates the condition. It is the UVA rays that are the issue I think.

We have read through researching that this condition can be caused because the porphyrins are not removed with dialysis because they are a large molecule.

I have also researched that N-Acetylcysteine may help with this condition. He is currently taking this antioxidant ( with the approval of his doctors) and has some improvement although he still gets the blisters, just not as many.

He will be on dialysis for the rest of his life. Well, until his liver actually fails and then they ‘might’ consider a liver/kidney transplant. So, sorting out the right dialysis for the rest of his life is very important to us.

In your opinion what do you think about HDF dailysis?
Does this form of dialysis help removed ‘large molecules’ from the blood?
Would it make a difference to porphyrin levels or not?

Would you have any ideas that you have tried or seen to help with this condition.

very concerned wife.
ps. thank you for sharing your time.

Dear Pauline:

I am sorry, but to be honest, I have no experience with dialysis of a patient with PCT or PPCT.

HDF does improve solute clearance - and this includes larger molecular solutes - but whether it would make sufficient difference … I do not know for sure.

There are some high porosity membranes available now (such as the high cut-off membranes we are now using for the removal of light chains in multiple myeloma) but I am not aware of these being used as yet for the treatment of such rare conditions as PCT or PPCT.

Peritoneal dialysis also permits much larger solute clearance and has been used (with a mixed bag of outcomes) in PCT but again, I do not have any personal experience here.

I will, when I get a moment, try to sift the literature a little to see if there are any pointers to better outcomes with such things as HDF, high cut-off membranes and/or PD … and ask around among some of my colleagues. If I find anything that might be useful, I will re-post here.

Sorry to have been such a dead loss to you on this one.

Thank you Dr Agar,

No need to be sorry, it isn’t a dead loss. I will also do some reading about High cut-off membranes and such things. We have never seen anyone with this condition either. I have read of three cases being treated with N-Acetylcysteine with success. But, it hasn’t worked very well so far for my husband…
PD isn’t an option for him.

thank you for your time I appreciate in advance any information you might find.

kind regards