If a dialysis
patient presents with a recurrent, sun-sensitive, bullous, and scarring rash on
their hands, think of an entity called “pseudoporphyria”.
Patients with ESRD have a limited capability to excrete porphyrins. If there is history of recurrent PRBCS transfusions and/or liver disease, accumulation of iron pigments happens, which in turn provoke pseudoporphyria, characterized by a photosensitive, vesiculobullous skin
eruption. Skin fragility and scarring are also observed.
See this reference
Pseudoporphyria is
clinically indistinguishable from porphyria cutanea tarda , and both conditions
will be associated with elevated serum levels of porphyrins. In the case of
pseudoporphyria, the elevated porphyrin levels result from lack of renal
excretion. There is no enzyme problem here like in true porphyria cutanea
tarda.
In addition to dialysis, a wide range of drugs are associated with pseudoporphyria. It was first identified in patients taking quinolones. NSAIDS, retinoids, diuretics and some anti
neoplastic agents such as TKIs have been associated with pseudoporphyria.
An entity to
monitor in our ESRD patients.
Picture courtesy: From an article in JAMA on cyclosporine induced pseudoporphyria.
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