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Leukocytoclastic vasculitis masking chronic vascular changes in previously undiagnosed erythropoietic protoporphyria.
[erythropoietic protoporphyria]
A
31
-
year
old
man
presented
with
swelling
and
purpura
of
the
dorsum
of
the
hands
following
sun
exposure
.
There
was
a
preceding
lifelong
history
of
photosensitivity
,
but
this
episode
,
which
occurred
after
the
recent
commencement
of
oral
iron
therapy
,
and
after
recent
alcohol
ingestion
,
was
much
more
severe
than
any
preceding
episode
.
Skin
biopsy
performed
48
h
after
the
onset
of
symptoms
showed
features
consistent
with
the
early
stages
of
leukocytoclastic
vasculitis
.
Direct
immunofluorescence
showed
homogeneous
thick
staining
of
the
vessel
walls
with
IgG
,
IgM
and
IgA
,
together
with
abundant
perivascular
fibrinogen
.
A
subsequent
periodic
acid-
Schiff
(
PAS
)
stain
on
the
skin
biopsy
revealed
thickening
of
the
walls
of
dermal
vessels
,
which
was
not
discernible
in
routinely
stained
(
hematoxylin
/
eosin
)
sections
.
The
diagnosis
of
erythropoietic
protoporphyria
(
EPP
)
was
confirmed
by
significantly
elevated
erythrocyte
protoporphyrin
levels
and
positive
plasma
fluorimetry
.
The
diagnosis
of
porphyria
may
have
been
missed
by
routine
skin
microscopy
if
not
for
the
additional
information
provided
by
clinical
history
,
direct
immunofluorescence
and
PAS
stain
.
The
pathogenesis
and
histopathology
of
acute
and
chronic
vascular
changes
in
EPP
are
reviewed
.
