November 2017 | Case of the Month

History

A 57 year old man was referred with an 18 month history of a blistering eruption on the backs of both hands. There were no obvious triggers for the blisters. There was personal or family history of skin problems. The patient had arthritis for which he took naproxen. He worked as a car mechanic, drank 12 units of alcohol a week, and had no relevant hobbies.


Examination

The patient had multiple erosions where the most recent blisters had been. There were no skin changes elsewhere. 


Investigations

What is the diagnosis?


Images

Diagnosis

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The diagnosis is pseudoporphyria.

Pseudoporphyria is a condition in which porphyria-like blistering of exposed skin on the extremities occurs in the absence of abnormal porphyrin metabolism. It can be caused by a number of drugs including naproxen (the case in this patient), furosemide, and nalidix acid. Once the drug is stopped the blisters will settle although this can take some time.

True porphyrias result from altered metabolism in the haem biosynthesis pathway, leading to an accumulation of porphyrins. There are three main types - porphyria cutanea tarda (PCT), variegate porphyria and erythrohepatic protoporphyria.

PCT is the most common type of porphryia, blisters result from a reaction between the porphyrins in the skin and UV radiation, which goes on to cause cell damage. Features of PCT include:

  • Although it is often worse in summer months the patient may not be able to clearly associate symptoms with sun-exposure
  • Clinical features include skin fragility and blisters on the backs of hands and bald areas of the scalp. Lesions heal slowly and often leave scars. Milia and areas of hyperpigmentation may develop.


Please follow the link to read more about porphyria.

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