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The cutaneous porphyrias.
[erythropoietic protoporphyria]
The
porphyrias
are
a
group
of
mainly
inherited
disorders
of
heme
biosynthesis
where
accumulation
of
porphyrins
and
/
or
porphyrin
precursors
gives
rise
to
2
types
of
clinical
presentation
:
cutaneous
photosensitivity
and
/
or
acute
neurovisceral
attacks
.
The
cutaneous
porphyrias
present
with
either
bullous
skin
fragility
or
nonbullous
acute
photosensitivity
.
This
review
discusses
the
epidemiology
,
pathogenesis
,
clinical
presentation
,
laboratory
diagnosis
,
complications
,
and
current
approach
to
porphyria
management
.
Although
focusing
mainly
on
their
dermatological
aspects
,
the
article
also
covers
the
management
of
acute
porphyria
,
which
by
virtue
of
its
association
with
variegate
porphyria
and
hereditary
coproporphyria
,
may
become
the
responsibility
of
the
clinical
dermatologist
.