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Parvovirus B19-associated catastrophic endothelialitis with a Degos-like presentation.
[malignant atrophic papulosis]
Parvovirus
B
19
is
a
single
-stranded
DNA
virus
exhibiting
affinity
for
a
variety
of
cell
types
including
endothelial
cells
.
The
basis
of
the
affinity
is
globoside
expression
,
the
receptor
for
B
19
.
B
19
endothelial
cell
parasitism
accelerates
endothelial
cell
apoptosis
,
potentially
critical
in
inducing
neoantigenicity
and
secondary
antibody
formation
.
A
38
-
year
-old
previously
healthy
male
developed
intestinal
and
cutaneous
infarction
,
elevated
creatinine
phosphokinase
(
CPK
)
levels
and
progressive
cytopenias
.
The
basis
of
his
clinical
presentation
was
unclear
until
a
skin
biopsy
suggested
a
potential
role
for
virally
mediated
endothelial
cell
injury
.
Hematoxylin
and
eosin
,
immunohistochemical
,
immunofluorescent
and
reverse
transcriptase
in
situ
polymerase
chain
reaction
studies
were
conducted
on
skin
biopsies
to
assess
for
viral
triggers
.
B
19
viremia
and
localization
of
B
19
RNA
transcripts
to
vascular
endothelium
were
uncovered
.
A
diagnosis
of
catastrophic
endothelial
cell
injury
attributable
to
B
19
infection
was
rendered
.
The
patient
showed
significant
improvement
with
intravenous
immunoglobulin
(
IVIG
)
.
Parvovirus
B
19
is
a
ubiquitous
virus
that
in
the
majority
of
affected
patients
remains
asymptomatic
.
Nevertheless
,
because
of
its
ability
to
infect
endothelium
it
can
cause
severe
multiorgan
endothelial
cell
injury
.
Diseases
Validation
Diseases presenting
"skin biopsies"
symptom
cadasil
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
fabry disease
harlequin ichthyosis
junctional epidermolysis bullosa
kindler syndrome
malignant atrophic papulosis
neonatal adrenoleukodystrophy
omenn syndrome
pyruvate dehydrogenase deficiency
sneddon syndrome
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