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Skin
and
Mucosal
Hemorrhage
of Prolonged
Systemic
By
A patient
JACOB
with
loidosis
is
AMuI,
primary
described
mucosal
ELENA
whom
hemorrhages
KESSLER
amyskin
clinical
manifestation
for
T
HE
clinically
PRESENTLY
remarkable
3 yr
as the
manifestation
The
disorder
was
the
sition
a 56-yr-old
housewife,
of
she
had
for
a year
noted
spontaneously
in
or
the
skin.
Physical
mouth,
values
platelet
functions
positive.
Serum
electrophoretic
normal.
erythrocyte
In
2 yr
April
paipated
ing
a
the
ESR,
in the
routine
ear,
tongue.
acetate
L light
left
axilla.
and
was
in
Extensive
showed
normal
morphology.
mucosa.
A skin
punch
changes.
The
excised
died in May 1969
Autopsy
showed
of the lower
lip.
biopsy
axillary
following
numerous
The
heart
first
and
emphasized.
about is
by
around
test
skin
punch
examinations,
because
of
A walnut
hemostatic
according
to
ml,
gIlOO
paper
and
Rumpel-Leede
and
a normal
a petechial
hemostatic
the
area
tests,
sudden
were
were
same
areas.
appearance
non-tender
studies
severe
ears,
electrophoresis,
in
the
sized
sensation
eyes,
2.7
petechiae
appearing
normal
hematological
of coagulation
factor
biopsy
from
including
protein
of
the
1966,
sfay
chin,
a peculiar
showed
studies
globulin
in
and
firm
as previously.
subcapsular
of
lymph
an
node
Follow-
hematomas
appeared
6.2 g/100
ml
showed
a dense
X-ray
lymph
features
admitted
mouth,
petechiae
serum
hour.
and
with
globulin
2.9 g/100
ml. Paper
and
band
in the gamma
globulin
region.
an IgG
“M”
protein
with
gamma
C-2
heavy
chains
studies
were negative.
A sternal
bone marrow
aspirate
Rectoscopy
from
preceded
fragility
examination,
demonstrated
chains.
and
appearance
Purpura
was
ears,
with
readmitted
the
throat
Serum
protein
electrophoresis
Immunoelectrophoresis
of
ml
continuous
patient
mm
,
are
first
eyes,
Laboratory
studies
mm.
Extensive
g/100
( ESR )
the
110
nose,
crops
acetate
cellulose.
A
regular
follow-up
for
depo-
Characteristic
purpura
children,
the
Capillary
6.7
rate
except
1968
fast
was
and
normal.
was
sedimentation
for
extremely
on the
cellulose
were
hemorrhagic
vascular
and withmucosal
systemic purpuraamyloidosis
for
healthy
scratching,
oral mucosa.
of 200,000/cu
protein
of the
REPORT
around
small
cause
disorder.
two
gentle
revealed
pattern
on
After
discharge,
of
spots
even
on the gums and
a platelet
count
was
normal
red
following
and
was
pinpoint
examination
and
with
mother
amyloid
VRIE5
widespread
of amyloid.
PATIENT
had
skin
of this
CASE
ES.,
ANDR#{201} DE
of 3 yr.
a period
REPORTED
in that
sole
AND
and
constituted
sole
in
Amyloidosis
systemic
in
Duration
revealed
a purpuric
gland
a cerebrovascular
showed
numerous
lesion
petechial
in the
slight
neck
reticular
hemorrhages
did
not
of
reveal
hyperplasia.
The
the
vascular
patient
accident.
hemorrhages
on the face and chin,
and a large
hematoma
weighed
320 g. The
liver
and
spleen
were
not
enlarged,
From Tel Aviv University Medical Center, Tel Aviv, Israel.
Submitted
September
18, 1970; revised December
19, 1970; accepted January 3, 1971.
JACOB
AMIR,
M.D.:
Chief
Physician, Department
of Medicine
D, Beilinson
Hospital,
Lecturer in Medicine, Tel Aviv University Medical
School, Tel Aviv, Israel. ELENA
KESSLER,
M.D.:
Deputy
Head,
J. Casper
Department
of Pathology, Beilinson Hospital;
Clinical
Lecturer in Pathology,
Tel Aviv University Medical School, Tel Aviv, Israel. ANDR#{201} DE
VinEs,
M.D.,
PH.D.:
Head
Department
crf Medicine
D, Bezlinson Hospital, Professor of
Medicine, Tel Aviv
University Medical School, Tel Aviv, israel.
530
BLOOD,
VOL.
37,
No.
5
(MAY),
1971
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JIEMOIIISI
lACE
Fig.
1000
intestine.
The
all
the
skin
of
the
lymph
in
blood
1966
70
Both
anivloid
in
the
larger
1968
were
Congo
vessels
1
and
of
2 )
present
in
the
and
the
in
were
(lerma
and
in
seen
were
heart,
tongue,
smaller
X 200.
stain.
deposits
1968
with
amyloid
stain
tissue.
Similar
reviews
connective
red
hemorrhages
Amyloid
softening.
blood
were
made
deposit.
Submucosal
of
( Figs.
deposits
iiopsy
walls and
aniyloid
with
respectively.
small foci
g,
gland
vessel
and
wall
and
muscles.
In
the
g
531
AMYLOIDOSIS
l)rain showed
particularly
organs,
skeletal
SYSTEMIC
1.-Arterial
weighing
in
IN
lungs,
vessels
were
1)100(1
vessel
walls.
revealed
amvloid
skin
biopsies
the
large
virtually
kidneys,
1)100(1
of
the
found
and
involved.
A
review
c1(’posits
in
obtaiiied
negative.
COMMENTS
Hemorrhages
loidosis
patient
yr prior
are
had skin
to other
Fig.
a common
may
and
and
rarely
P1rIMlra
manifestations
sanie
vessel
as
mucosal
and
its
mucosal
clinical
2.-The
cutaneous
precede
other
clinical
as an isolated
of amvloidosis.
in Fig.
1 viewed
manifestation
phenomenon
under
of amyOur
symptomatology.7
polarized
for
light.
about
3
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532
AMIR,
Amvloid
on
purpura
the
face,
is preferably
neck,
and
localized
upper
in the
extremities,7
upper
and
KESSLER
part
often
AND
of the
on
the
DE
body,
VRIES
mainly
eyelids
and
the
sides of the
elicited
by
hemorrhages
nose.3
The lesions,
which
may appear
spontaneously,8’9
may also be
external
pressure8’1#{176} and
even
by gentle
scratching.7’1’
Mucosal
in amyloidosis
occur
mainly
in the mouth
and rectum,
but may
as gross
gastrointestinal
bleeding.3
In our patient,
as in others
re-
manifest
the
ported,7’1’5
rhages
in
glossia,
a common
In
the
a few
dency
sequel
of
with
been
the
vessel
Such
the
purpura
wall,
invoked
in this
systemic
the
the
of
to
the
of
macro-
the
hemorrhagic
ten-
abnormalities,
V and
Vu,’8
were
not
such
fibrinolysis,’9
detected
and
in
paraproteinemia.
furnish
hemor-
absence
tongue.’
amyloidosis
pathogenesis
appears
traumatic
the
blood-clotting
in factor
preceded
and
in
involvement
with
in
spontaneous
occurred
abnormalities
amyloidosis,2’23
iurpura
amyloid
decrease
fibrinogeiiemia.’8’2#{176}
the
these
associated
X deficiency,16”7
systemic
were
primary
found
Furthermore,
in
features
Remarkably,
patients
has
factor
striking
tongue.
our
Amyloid
of
the
a
sufficient
as
hypopatient.
deposition
hemorrhagic
tendency
in
explanation
of
the
in
iri-
patient.
REFERENCES
1. Eisen,
loidosis.
2.
C.
55:943,
Coltz,
3.
dosis.
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a
an
of
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genetic
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Knorp,
\V.
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1945.
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E.
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and
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1968.
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C. C., Waldmann,
Th. A., and Zellis,
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K.,
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M.:
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X (ICficiency
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amyloidosis:
report
J.:
P.,
primary
(Basel)
19.
loidosis
C.,
case.
Blood
Vorlova,
hemorrhagic
Unusual
ski,
of
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cal
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and
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89:678,
:
18.
Ass.
J.,
hemorrhage
systemic
12.
I. A.
amyloidosis
1961.
9. Cohen,
progress.
Primary
with
associated
H.
intralesional
mars’
to
and
clinical
prognosis
D.,
amyloidosis.
K.,
A
W.
Carey,
J. H., and
systemic
amyloidosis:
39:315,
Brandt,
S.:
Block,
familial
systemic
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cases.
F.,
29
S.,
(Chicago)
two
1956.
Lindsay,
mary
of
experimental,
the
35:239,
mucous
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J.,
H.
and
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on
(Bait)
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amyloi-
skin
31:381,
study
emphasis
the
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review
clinical
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of
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E.,
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Amyloidosis.
W.:
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lesions
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Briggs,
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HEMORRhAGE
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Higgins,
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with
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Atypical
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From www.bloodjournal.org by guest on March 4, 2016. For personal use only.
1971 37: 530-533
Skin and Mucosal Hemorrhage of Prolonged Duration in Systemic
Amyloidosis
JACOB AMIR, ELENA KESSLER and ANDRÉ DE VRIES
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