Elephantiasis in the Netherlands, a rare finding and a reason to perform an autopsy

Abstract

Introduction: Elephantiasis is endemic around the equator. In Western countries the phenomenon is extremely rare. Case description: A Dutch 41-year-old severely obese male was found dead in his home. During the external examination, an extensive skin infection was seen on both lower legs. The man was treated for erysipelas two years prior and did not visit the general practitioner again. Clinical autopsy showed there was a serious stasis dermatitis of both lower legs with a superimposed erysipelas which led to a sepsis and multiple organ failure. The erysipelas presented itself as elephantiasis. Discussion: Elephantiasis is most often caused by filariasis. In our case, the deceased had not been abroad and had predisposing factors for elephantiasis nostras verrucosa (ENV), such as obesity and an objectified erysipelas infection two years prior. The chronic skin infection caused sepsis and multiple organ failure. The position in which the deceased was found may have contributed to the death. Conclusion: Elephantiasis is a highly uncommon phenomenon in Western countries. A chronic erysipelas can cause non-filarial elephantiasis and if left untreated, can cause death.

Full text

Forensic
Pathology
Elephantiasis
in
the
Netherlands,
a
rare
finding
and
a
reason
to
perform
an
autopsy
H.
Tamara
Gelderman
a
,
Jos
Bart
b
,
Wilma
L.J.M.
Duijst
a,c,
*
a
PHS
IJsselland,
Zeven
Alleetjes
1,
8011
CV
Zwolle,
the
Netherlands
b
Isala
Hospital,
Dokter
van
Heesweg
2,
8025
AB
Zwolle,
the
Netherlands
c
Maastricht
University,
Faculty
of
Law
and
Criminology,
Minderbroedersberg
4-6,
6211
LK
Maastricht,
the
Netherlands
A
B
S
T
R
A
C
T
Introduction:
Elephantiasis
is
endemic
around
the
equator.
In
Western
countries
the
phenomenon
is
extremely
rare.
Case
description:
A
Dutch
41-year-old
severely
obese
male
was
found
dead
in
his
home.
During
the
external
examination,
an
extensive
skin
infection
was
seen
on
both
lower
legs.
The
man
was
treated
for
erysipelas
two
years
prior
and
did
not
visit
the
general
practitioner
again.
Clinical
autopsy
showed
there
was
a
serious
stasis
dermatitis
of
both
lower
legs
with
a
superimposed
erysipelas
which
led
to
a
sepsis
and
multiple
organ
failure.
The
erysipelas
presented
itself
as
elephantiasis.
Discussion:
Elephantiasis
is
most
often
caused
by
filariasis.
In
our
case,
the
deceased
had
not
been
abroad
and
had
predisposing
factors
for
elephantiasis
nostras
verrucosa
(ENV),
such
as
obesity
and
an
objectified
erysipelas
infection
two
years
prior.
The
chronic
skin
infection
caused
sepsis
and
multiple
organ
failure.
The
position
in
which
the
deceased
was
found
may
have
contributed
to
the
death.
Conclusion:
Elephantiasis
is
a
highly
uncommon
phenomenon
in
Western
countries.
A
chronic
erysipelas
can
cause
non-filarial
elephantiasis
and
if
left
untreated,
can
cause
death.
A
R
T
I
C
L
E
I
N
F
O
Keywords:
Filariasis
Elephantiasis
nostras
verrucosa
Erysipelas
Introduction
The
term
elephantiasis,
or
elephant
skin,
refers
to
an
enlarged
body
part
as
a
result
of
chronic
lymphedema.
Filariasis,
a
tropical
disease,
is
the
most
common
cause
of
elephantiasis
worldwide.
Filariasis
is
called
a
disease
of
the
poor
and
it
is
a
severe
public
health
problem.
Filariasis
is
endemic
in
more
than
80
countries.
Currently,
around
120
million
people
are
infected,
of
which
43
million
have
swelling
of
the
limbs
and/or
breasts.
Another
120
million
people
are
at
risk
of
getting
the
infection.
The
Global
Program
for
the
Elimination
of
Lymphatic
Filariasis
(GPELF)
was
established
in
2000.
Worldwide
there
are
more
than
60
million
filariasis
programmes
in
34
countries
around
the
equator
[1].
An
infection
with
filariasis
originates
after
a
human
individual
is
infected
by
the
filarial
parasite,
which
is
transported
by
mosquitoes.
Filariasis
is
caused
by
three
different
roundworms:
W.
Bancrofti,
B.
Malayi
and
B.
Timori,
which
are
found
in
tropical
and
subtropical
areas
[2,3].
The
worms
nestle
in
the
lymph
vessels,
causing
them
to
malfunction.
The
infection
can
be
asymptomatic,
acute
or
chronic.
An
asymptomatic
infection
causes
damage
to
the
lymph
system,
but
has
no
symptoms.
A
chronic
infection
causes
lymphedema
or
elephantiasis
of
the
limbs.
There
is
usually
involvement
of
the
genitals.
Acute
episodes
often
occur
beside
the
chronic
situations
because
the
protective
skin
layer
is
disrupted.
Elephantiasis
is
treated
with
medications
such
as
albendazol
or
ivermectine
[4].
Filariasis
is
treated
with
diethylcarbamazine
(DEC).
DEC
can
be
taken
in
combination
with
albendazole.
The
combination
has
shown
good
long-term
results
[1].
Other
forms
of
elephantiasis
are
seen
in
breast
cancer
(lymphedema
of
the
arm),
Leishmania
(disseminated
spots
on
the
body)
and
Lymphogranuloma
venereum
(lymphoma
of
the
groin).
Non-filarial
elephantiasis
of
the
legs
is
called
elephantiasis
nostras
verrucosa
(ENV)
and
results
from
the
lymphatic
blockage
of
the
lymphatic
system.
The
fibrosis
can
be
caused
by
recurrent
bacterial
skin
infections
or
by
non-
infectious
causes.
Characteristics
are
non-pitting
edema,
hyperkeratosis
of
the
dermis,
papillomatosis
and
verrucous
lesions,
causing
the
appearance
of
an
elephants
leg
[2].
Stasis
dermatitis
is
usually
caused
by
chronic
venous
insufficiency
[5].
Obesity
is
a
predisposing
factor,
because
it
reduces
lymph
drainage
and
aggravates
venous
insufficiency
[2].
The
first
stage
is
characterised
by
edema
with
a
shiny
and
erythematous
appearance.
Afterwards
dry,
scaly
spots
or
spots
with
ulceration,
crusts
and
moisture
discharge
arise
[6].
The
skin
is
damaged,
which
causes
a
higher
risk
of
erysipelas
or
cellulitis
[7].
The
skin
infections
are
caused
by
beta-hemolytic
streptococci
of
which
group
A
is
the
most
common.
An
infection
with
Staphylococcus
Aureus
*
Corresponding
author
at:
Minderbroedersberg
4-6,
6211
LK
Maastricht,
the
Netherlands.
E-mail
addresses:
t.gelderman@ggdijsselland.nl
(H.
T.
Gelderman),
J.bart@isala.nl
(J.
Bart),
wilma.duijst@maastrichtuniversity.nl
(W.L.J.M.
Duijst).
http://doi.org/10.1016/j.fsir.2020.100056
Received
30
September
2019;
Received
in
revised
form
16
December
2019;
Accepted
2
January
2020
Available
online
10
January
2020
2665-9107/©
2020
The
Authors.
Published
by
Elsevier
B.V.
This
is
an
open
access
article
under
the
CC
BY-NC-ND
license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
Forensic
Science
International:
Reports
2
(2020)
100056
Contents
lists
available
at
ScienceDirect
Forensic
Science
International:
Reports
jo
u
r
nal
h
o
mep
ag
e:
ww
w.els
evier
.c
om
/lo
cat
e/fs
ir

is
less
common
[8].
Eventually,
the
area
becomes
chronically
colonised
by
multiple
species
of
bacteria
and/or
fungi.
Lymph
stasis
can
be
managed
by
applying
compression
stockings
and
mechanical
massages.
Elastic
bandage
compression
is
reported
to
be
an
effective
treatment.
Diuretics
and
systemic
antibiotics
can
be
used
to
reduce
edema.
Antibiotics
can
be
given
to
treat
the
infection.
To
treat
the
hyperkeratotic
plaques,
topical
keratolytics
or
systemic
retinoids
might
be
necessary.
When
using
retinoids,
monitoring
of
serum
lipids
and
liver
function
is
needed.
In
advanced
stages
the
outcome
of
treatment
of
ENV
is
poor
[9].
The
expected
survival
for
a
patient
with
ENV
is
based
on
the
severity
of
lymphedema,
underlying
diseases,
and
other
contributing
factors.
Early
diagnosis
and
early
intervention
to
disrupt
the
vicious
cycle
will
render
a
better
outcome.
There
are
no
articles
found
with
exact
frequency
numbers
of
ENV.
Worldwide
there
are
a
couple
of
case
reports
published
stating
that
elephantiasis
after
recurrent
erysipelas
is
very
rare
[10,11].
This
is
the
first
case
observed
(and
described)
in
the
Netherlands.
Case
report
A
41-year-old
Caucasian
man
was
found
dead
in
his
home
by
his
brother.
He
was
lying
naked
on
his
stomach
in
the
bathroom.
During
the
external
investigation,
performed
by
a
forensic
physician
and
forensic
researchers,
the
following
findings
were
observed:
-
There
were
no
signs
of
burglary
or
a
struggle.
-
He
was
lying
in
the
prone
position
with
his
face
against
the
floor
and
his
hands
beside
his
head
(arms
stretched
out
to
the
side).
-
He
was
severely
obese,
estimated
length
1.90
m
and
weight
200
kg
(estimated
BMI
55
kg/m
2
).
-
There
were
petechia
on
the
nose,
on
the
eyelids
and
above
the
right
eyebrow.
-
The
tongue
protruded
from
the
mouth.
There
were
no
abrasions
of
the
tongue.
-
The
livor
mortis
disappeared
completely
on
thumb
pressure
and
the
rigor
mortis
was
in
the
beginning
stage.
-
No
bruises
or
abrasions
were
present.
-
There
was
no
indication
that
fractures
were
present.
-
In
the
skin
folds
between
the
abdomen
and
the
groin,
skin
abnormalities
fittingwith skin infections (erysipelas)and
a
rough,
slightlycobblestoned
skin
were
found.
-
Both
lower
legs
were
swollen
and
showed
an
extremely
rough,
cobblestoned
skin,
starting
from
the
knee
up
to
the
toes.
At
the
proximal
part
of
the
lower
legs
a
white
circular
debris
(width
5
cm)
was
seen.
The
right
lower
leg
showed
epidermolysis,
with
wet
areas
with
greenish
mash,
haemorrhagic
areas
and
blisters.
The
left
lower
leg
showed,
proximal
from
the
knee,
multiple,
little,
dark-red
to
black
coloured
maculae.
The
spots
did
not
disappear
on
pressure
and
they
presented
as
purpura
or
petechia
(Photos
1–4).
-
On
the
left
hip
and
buttock
there
was
a
region
showing
multiple,
little
(0.1
cm),
dark-red
to
black
coloured
maculae.
The
spots
did
not
disappear
on
pressure
and
they
presented
as
purpura
or
petechia
(Photo
5).
According
to
the
family,
the
deceased
had
not
been
abroad.
Until
recently
he
had
been
going
to
his
place
of
work.
He
rarely
visited
the
general
practitioner
or
other
healthcare
providers.
The
general
practitioner
last
saw
the
deceased
two
years
ago
because
of
an
erysipelas
infection
of
both
the
lower
legs.
There
had
been
no
contact
afterwards
with
the
general
practitioner
and
it
was
unknown
whether
the
treatment
was
successful.
Because
there
were
no
signs
of
burglary,
no
signs
of
a
struggle
and
no
signs
of
violence
on
the
body,
a
criminal
offence
was
not
thought
to
be
Photo
1.
Lower
legs
and
feet.
The
green
arrows
show
the
crusts.
The
yellow
arrow
shows
the
fungal
colonisation.
Photo
2.
Right
foot.
Photo
3.
Detail
right
calf.
Photo
4.
Detail
left
calf/ankle.
H.T.
Gelderman
et
al.
FSIR
2
(2020)
100056
2

probable.
Because
the
deceased
had
attended
work
recently
and
had
not
mentioned
any
medical
problems,
a
natural
cause
of
death
was
not
found
to
be
probable.
Because
of
the
extreme
obesity
and
the
prone
position
in
which
the
body
was
found,
the
cause
of
death
was
thought
(by
the
forensic
physician)
to
be
positional
asphyxiation
possibly
after
the
man
lost
his
balance.
We
thought
he
may
not
have
been
able
to
stand
up
from
this
position
because
of
his
weight.
The
petechia,
visible
in
the
face
and
occurring
after
asphyxia,
support
this
hypothesis.
The
passing
was
registered
as
an
unnatural
death.
In
consultation
with
the
family,
a
clinical
autopsy
was
performed.
The
results
of
the
autopsy:
the
heart
(830
gr)
showed
concentric
myocardial
hypertrophy
of
the
left
ventricle
with
signs
of
hypoperfusion.
There
was
extensive
peripheral
edema
and
stowage
of
blood
in
the
lungs,
liver,
spleen
and
kidneys,
fitting
with
cardiac
decompensation.
The
kidneys
showed
necrosis
of
the
proximal
tubule
cells.
The
spleen
was
enlarged
and
showed
a
disorganized
parenchyma
with
abnormal
white
and
red
pulpa.
The
adrenal
glands
were
of
normal
size
and
showed
a
normal
cortex
and
medulla.
The
bone
marrow
showed
a
left
shift
of
the
white
blood
cells,
fitting
with
a
severe
acute
inflammation.
Macroscopically,
the
skin
of
both
lower
legs
showed
extreme
swelling
of
the
lower
legs
up
to
the
toes
with
a
cobble
stone
effect.
After
cleaning
of
the
body,
several
infiltrations
of
the
skin
were
seen.
Microscopically
extensive
scar
formation
with
chronic
inflammation
infiltrates,
fitting
with
stasis
dermatitis
with
an
acute
inflammation
with
necrosis
of
the
dermal
collagen
(Photos
6–8)
was
seen.
The
inflammation
reached
focally
into
the
deep
dermis.
The
additional
microbiological
examination
of
a
skin
biopsy
of
both
lower
legs
and
a
smear
test
of
the
skin
lesions
of
the
hip
showed
the
presence
of
Staphylococcus
Aureus
and
haemolytic
Streptococci
group
G.
In
conclusion,
there
is
a
serious
stasis
dermatitis
of
both
lower
legs
with
a
superimposed
erysipelas
which
led
to
a
sepsis
and
multiple
organ
failure
(in
particular
of
the
heart,
lungs
and
kidneys).
Discussion
The
rough
cobblestoned
skin
as
observed
in
the
deceased
can
be
labelled
as
elephantiasis.
Microscopically
the
lymphatic
stasis
was
confirmed.
The
elephantiasis
was
in
a
stage
of
chronic
inflammation,
ulceration
and
colonisation
with
bacteria
and
supposedly
fungi.
The
man
had
not
been
abroad
which
means
that
filariasis
could
be
ruled
out.
The
Photo
5.
Left
hip/buttock.
The
green
arrow
shows
the
petechia.
Photo
6.
Overview
stasis
dermatitis.
Black
arrow:
stratum
corneum.
Blue
arrow:
epidermis.
Red
arrow:
dermis.
Black
oval:
edema.
Black
square:
detail
Photo
7.
Photo
7.
Detail
stasis
dermatitis.
Black
arrow:
macrophage.
Blue
arrows:
lymphocytes.
Green
arrows:
endothelial
cell
nuclei.
H.T.
Gelderman
et
al.
FSIR
2
(2020)
100056
3

man
was
obese
and
had
an
erysipelas
infection
of
his
legs
which
was
treated
two
years
prior.
No
medical
check
had
been
done
since
the
treatment
of
the
infection.
The
microbiological
examination
of
a
skin
biopsy
and
a
smear
test
of
the
skin
lesions
of
both
lower
legs
showed
the
presence
of
Staphylococcus
Aureus
and
haemolytic
Streptococci
group
G,
which
points
at
a
bacterial
infection
of
the
lower
legs
disseminating
to
the
hip
(visible
via
the
petechia).
The
fungi
were
not
objectified
microscopically.
It
is
not
clear
whether
the
infection
was
treated
adequately
two
years
before
and
whether
the
symptoms
had
disappeared
in
the
meanwhile.
Because
of
the
severity
of
the
elephantiasis
and
the
infection,
it
most
probably
had
not.
The
conclusion
of
this
case
is
that
the
man
died
of
ENV
caused
by
a
chronic
erysipelas
infection
and
obesity,
complicated
by
a
sepsis
and
multiple
organ
failure.
The
hypoperfusion
of
the
heart,
cardiac
decomposition
and
necrosis
of
the
proximal
tubule
cells
in
the
kidneys
fit
with
this
conclusion.
The
obesity
of
the
deceased
and
the
prone
position
in
which
he
was
found
could
have
contributed
to
death.
Conclusion
Elephantiasis
is
most
often
caused
by
filariasis,
a
tropical
disease.
Non-
filarial
elephantiasis
can
be
the
result
of
a
chronic
erysipelas
infection
which
can
lead
to
sepsis,
multiple
organ
failure
and
death
if
not
treated
in
time.
Although
non-filarial
elephantiasis
is
highly
uncommon
in
Western
countries,
it
does
occur
and
should
be
considered
as
a
cause
of
death
in
an
otherwise
unexplained
death.
Declaration
of
Competing
Interest
None.
References
[1]
D.
Molyneux,
Lymphatic
Filariasis
(Elephantiasis)
Elimination:
a
Public
Health
Success
and
Development
Opportunity,
(2003)
http://www.filariajournal.com/
content/2/1/13.
[2]
K.
Sisto,
A.
Khachemoune,
Elephantiasis
nostras
verrucosa:
a
review,
(Am.
J.
Clin.
Dermatol.
9
(3)
(2008)
141–146.
[3]
WHO,
Lymphatic
Filariasis
(elephantiasis):
546
Million
People
Treated
Worldwide
in
2007
Alone,
WHO,
2011https://www.who.int/neglected_diseases/
integrated_media_lf/en/.
[4]
Lymphatic
Filariasis,
(2019)
(n.d.)
https://www.who.int/news-room/fact-sheets/
detail/lymphatic-filariasis
(accessed
September
16,
2019).
[5]
S.
Sundaresan,
M.R.
Migden,
S.
Silapunt,
Stasis
dermatitis:
pathophysiology,
evaluation,
and
management,
(Am.
J.
Clin.
Dermatol.
18
(2017)
383–390,
doi:http://
dx.doi.org/10.1007/s40257-016-0250-0.
[6]
R.A.
Weedon,
G.
Strutton,
Weedon’s
Skin
Pathology,
7th
ed.,
Churchill
Livingstone,
Edinburgh,
2010.
[7]
J.V.
Hirschmann,
G.J.
Raugi,
Lower
limb
cellulitis
and
its
mimics:
part
II.
Conditions
that
simulate
lower
limb
cellulitis,
(J.
Am.
Acad.
Dermatol.
67
(2012)
177,
doi:http://
dx.doi.org/10.1016/j.jaad.2012.03.023
e1-177.e9.
[8]
Cellulitis
and
Skin
Abscess:
Clinical
Manifestations
and
Diagnosis
-
UpToDate,
(2019)
(n.d.).
https://www.
uptodate.com/contents/cellulitis-and-skin-abscess-
clinical-
manifestations-and-diagnosis?search=
cellulitisandskinabscess&source=search_
result&selectedTitle=2150&usage_type=default&display_rank=2
(accessed
September
16,
2019).
[9]
D.
Baird,
D.
Bode,
T.
Akers,
Z.
DeYoung,
Elephantiasis
Nostras
Verrucosa
(ENV):
a
complication
of
congestive
heart
failure
and
obesity,
(J.
Am.
Board
Fam.
Med.
23
(2010)
413–417,
doi:http://dx.doi.org/10.3122/jabfm.2010.03.090139.
[10]
A.
Freitas,
J.F.M.
Rodrigues,
Elephantiasis
nostras
verrucosa
secondary
to
recurrent
erysipelas,
(BMJ
Case
Rep.
2017
(2017)
,
doi:http://dx.doi.org/10.1136/bcr-2017-
221014.
[11]
Y.P.
Yang,
W.X.
Huang,
W.X.
Zhong,
Y.M.
Fu,
P.A.
He,
G.
Zhao,
Q.M.
Feng,
Bilateral
lower
limb
and
abdominal
elephantiasis
due
to
erysipelas,
(Chin.
Med.
J.
(Engl.)
131
(2018)
873–874,
doi:http://dx.doi.org/10.4103/0366-6999.228244.
Photo
8.
Overview
erysipelas
and
pustel.
Black
oval:
purulent
inflammation.
Red
oval:
dermal
interstitial
inflammation.
Blue
oval:
dermal
perivascular
lymphocytic
infiltrate.
Black
arrow:
stratum
corneum.
Blue
arrow:
epidermis.
Red
arrow:
dermis.
H.T.
Gelderman
et
al.
FSIR
2
(2020)
100056
4