Now let's talk about porphyria cutanea tarda.
As I mentioned before, all the porphyrias
are essentially derived or caused by
a metabolic disorder due to the
disruption of the normal heme
biosynthesis pathways. And this is just
a representative picture of the heme
molecule. And there's a variety
of steps that can occur
along the way to creating a heme molecule.
There are both inherited and
also acquired deficiencies
in the pathways that lead to heme, and
in this case, porphyria cutanea tarda
involves an inherited or acquired
deficiency of urogen decarboxylase.
Most common age of symptom
onset is around 30-50 years of age,
and flares can be precipitated at any time.
Oftentimes, by certain medications
that a person ingests,
alcohol has been shown to be
a trigger, minor skin trauma,
and then sun exposure, and that's
why we'll oftentimes see these
Patients who have this disease
will complain of fragile skin,
feeling pruritic, they may have
vesicles can occur, bullae, and
there can certainly be scarring
after repeated flares over
the span of many years.
As I mentioned, the face,
the back of the hands,
and there's also this predilection for
hypertrichosis or increased hair
in areas of the face.
This is a representative lesion of some of
the findings you may see in the skin.