00:01
What or how does one get syphilis?
Well, I think most people probably are aware of that but
syphilis
as a disease occurs in several different stages and the
transmission or the contagiousness of the disease
depends in part on at what stage the patients is in their
syphilis progression.
00:22
In the primary stage, the very first set of disease
manifestations, sexual contact is the principal mode of
transmission.
00:30
There are many treponemes or many Treponema pallidum
organisms
present at the mucosal surfaces in the vaginal mucosal
walls,
in the semen at the tip of the penis - all of these are very
contagious
and easily transmissible to other sexual partners.
00:48
The image you see on this slide shows again, in beautiful
living color, the treponemes,
done in sort of an artist rendition along with some of the
epithelial cells seen in vaginal mucosa.
01:01
In the secondary stage of syphilis - so after some
dissemination has occurred,
then any lesions any of the rashes which we’ll discuss in
just a minute,
are highly contagious because they too are filled with
disseminated Treponema pallidum.
01:18
And mothers at any stage but especially in the first and
second stages can transmit Treponema pallidum,
can transmit the causative agent of syphilis to their unborn
babies creating congenital syphilis
and this too has a specific set of clinical findings which
we’ll cover very shortly.
01:38
And then finally transfusion of blood from the patient who
is actively infected with syphilis also can transmit the
process.
01:46
So Treponema pallidum quite wily in escaping immune
recognitions and quite wily at creating transmission
via highly contagious skin findings and other contagious
lesions to create additional victims.
02:00
So, before we even talk about the stages of syphilis, let’s
talk about what we can do to prevent them.
02:08
Well, if the most likely mode of acquisition is being
exposed to mucosal surfaces or to genital surfaces,
then practicing barrier contraception via condoms
is the most effective way of preventing transmission of
syphilis.
02:23
Treatment however for those patient who have forgotten
or been unable to avoid acquiring Treponema pallidum, is
with penicillin;
a lot of penicillin typically injected as a shot into the
buttocks and it is a painful injection.
02:40
Talk about paying a penalty for having one night of fun.
02:44
Some patients pay an even higher penalty.
02:46
This is when they develop a reaction called the
Jarisch-Herxheimer reaction
in which the treatment of the Treponema pallidum kills the
bacteria and releases
so much antigen so much external toxin from the cell wall
that it further induces
an intense inflammatory reaction. Patients with the
Jarisch-Herxheimer reaction
will have high fevers, shaking chills or rigors and many
times hypotension or even shock.
03:16
So now, without further ado, as I promised several slides
ago,
let’s talk about the stages of syphilis and we’ll start with
primary syphilis,
the first indication of a clinical response to infection
with Treponema pallidum.
03:31
Patients with primary syphilis will may times have chancres,
these are painless ulcerative lesions on the genitalia.
03:39
In here you can see a picture of the shaft of the penis
and although it doesn’t show as well there are also some
early lesions on the glands penis.
03:48
These lesions can occur throughout the genitalia and the
perineal surfaces
meaning around the vagina, around the anus.
03:55
They’re ulcerative lesions, typically with some amount of
surrounding erythema or redness
but they are painless, that’s the key thing.
04:04
Proximal to or draining from the site of the chancres, will
be inflamed lymph nodes, buboes,
very much like we have described in our other session about the
plague with you Yersinia pestis.
04:18
Buboes are painless but swollen lymph nodes occurring
several weeks
after the initial infection occurs and they simply suggest
that’s where the major immune reaction is occurring.
04:31
Where do you typically see signs of primary syphilis?
Well, typically at site of the inoculation so external
genitals as we said, the anus,
the vagina or sometimes even the mouth.
04:45
Onset, up to three weeks after initial introduction of the
Treponema,
21 days is actually fairly accurate and healing starts for
the next 3 to 6 weeks afterwards
but it can be a slow process.
05:00
At this stage, anytime during the incubation period
certainly during the signs and presentation of clinical
symptomatology,
one can make a diagnosis through dark field microscopy
looking at a swab from the base
of one of these lesions, yes, it is somewhat exciting to
acquire that
but it’s how one can make the diagnosis.
05:19
One can also start to look for the serologic responses with
the non-Treponemal
and Treponemal tests. Treponemes certainly we found in
chancre fluid
if possible or even potentially in draining one of those
buboes, those inflamed lymph nodes.
05:35
And if one obtains the non-Treponemal test, the VDRL or the
RPR, those will be positive
and up to 80% of patients but keep in mind that just as
false positives occur so too do false negatives.
05:51
So a negative test in this situation does not exclude
primary syphilis.
05:55
Moving on then to secondary syphilis, here the clinical
manifestations are very much a flu-like illness.
06:03
It’s not specific, there may be low to sometimes high grade
fevers.
06:07
The patients feel quite ill, they have anorexia, they have
sore muscles with myalgias.
06:14
They have swollen lymph nodes in a generalized way so
generalized lymphadenopathy.
06:18
Certainly they might have things like sore throat, headache
if they didn’t know any better
and many patients don’t, because their primary syphilis may
have been silent,
they might simply think they have the flu.
06:30
However development of the rash is starting to become a very
specific sign of secondary syphilis.
06:36
The rash as described in the slides is a disseminated
maculopapular rash
and you can see two examples of that in the top two images
on the slide,
the one that shows the back, flat somewhat erythematous
lesions which you're noticing.
06:51
The upper right however shows the hallmark of a syphilitic
rash in the secondary stage
that is presence of lesions on the palms and on the soles of
the feet.
07:02
There are very few infections known to human medicine, human
science that cause a palmar
or plantar rash and secondary syphilis is absolutely one of
them.
07:13
If one then finds a third finding, so flu-like illness,
palmar or plantar rash along with a disseminated
maculopapular rash and this third finding called Condyloma
latum,
these are flat lesions they look very much wart-like and
that would be the bottom picture on the slide.
07:32
They’re smooth, they are moist they typically occur again at
the scene or the site of initial inoculation,
so the perivaginal area, the perirectal area, sometimes
around the glans penis.
07:45
These along with the palmar and plantar rash should be
screaming the diagnosis of secondary syphilis.
07:52
And as we’ve noted, the rashes and the lesions will easily
be found in the anogenital area.
07:59
Onset. This typically occurs sometimes days to weeks to
months rarely years after the primary syphilis starts.
08:10
The most typical time is four to ten weeks after the primary
infection occurs
and it lasts up to eight months or so.
08:19
The lesions themselves are highly contagious.
08:22
This is unfortunate because many patients of course will not
recognize the significance of a palmar
or plantar rash and as you, the unsuspecting clinician
walk into the room
to introduce yourself to the patient appropriately shaking
hands,
you may have just been exposed to millions upon millions of
Treponema pallidum
transferred by a simple hand shake. Unfortunate, but yet we
are physicians
and therefore very good hosts - sorry.
08:50
After resolution, then there’s another quiescent period
before we get to the next stage
but during any point of the stage, the active secondary
syphilis or even as the lesions
are resolving one can demonstrate Treponema pallidum through
dark field microscopy
and of course with the serologic tests which we’ve already
talked about.
09:10
Moving then on to the final stage, tertiary syphilis.
09:15
Tertiary syphilis has a been associated with such lesions as
called gummas
which is represented in the scrotum of the patient in the
picture on the slide.
09:27
Gummas are granulomatous lesions but they are very slow
growing and they are very slowly destructive.
09:33
In fact, because they’re relatively asymptomatic, other than
looking quite noxious
if they’re on the surface of the skin, they can cause
hollowed out or pitted lesions,
especially in bone and connective tissue.
09:46
In looking at archeologic specimens in patients from certain
parts of the world
where syphilis was quite prominent, one can see these
hollowed out pits or cavities
in the flat bones and even the long bones of those patients
suggesting that syphilis
is very much disease of the old world. Columbus wasn’t the
only one who was associated with syphilis.
10:10
Also present in this tertiary stage of syphilis can be very
severe systemic manifestations,
one of which is neurosyphilis.
10:20
This is a very bad player because it can create a
demyelinating or inflammatory lesion of the dorsal horns
and posterior columns of the spinal cord and it can be
slowly progressive,
so patients may start with loss of sensation, they may start
to be ataxic in their gait.
10:39
They may develop a broad based gait which has also been
called sometimes a sailor’s gait like a sailor on a ship.
10:47
They also may develop the Argyll Robertson pupil which has
been unfortunately nicknamed the prostitute's pupil.
10:55
This is the pupil that when you ask the patient to look afar
and then up close,
their pupil does constrict with accomodation but it does not
react to light, the afferent light reflects
has been systematically destroyed by neurosyphilis.
11:09
Patients may also developed cardiovascular syphilis in which
they can get inflammation
any of the blood vessels but especially the aorta along with
aortic dissection
and aortic aneurysmal rupture, not a good player at all.
11:23
So you can see a variety of signs under the clinical
manifestations described for tertiary syphilis.
11:29
The broad base, the ataxia, as noted for neurosyphilis;
having a positive Romberg sign meaning that they have
ataxia,
they’re unbalanced, developing Charcot joints along with
contractures.
11:42
Even having stroke without hypertension - due to the
aortitis, due to the vascular inflammation.
11:49
So, this process after the secondary syphilis can take years
to decades and it’s not everybody
but in effect it may not be anybody for even decades until
they eventually pass away,
but in those few who do develop tertiary syphilis, it is
quite a diagnostic dilemma
because most of these signs are nonspecific and most of
these signs are hidden under the surface of the skin
so diagnosis can still be made serologically with the
non-Treponemal
and the Treponemal antibodies as well as performing
specialized molecular diagnostics
such as the PCR on spinal fluid, but it takes diagnostic
suspicion
to think of tertiary syphilis before one even sends the
proper tests.
12:34
Let’s spend a little time talking about congenital syphilis
mostly
because this is about as frequent as are the primary and
secondary syphilis cases
we just discussed with adults in large part because mothers
who may be carrying child
may not be aware that they have a primary or secondary
syphilis.
12:53
Transmission can occur at any stage of syphilis.
12:58
It occurs most clinically significantly in terms of the
fetus, the baby,
when it occurs during the first trimester and this makes
sense because infection
and transmission by any infectious agent will always have
greater impact the earlier in the life of the fetus that it
occurs.
13:16
It can affect tissue progress, it can affect tissue
development,
so that then is the time to test the mothers; the earlier
the better
and in fact, most parts of the states in most parts of the
world,
recommend testing pregnant mothers at least twice and in
fact three times during the pregnancy.
13:35
The first time upon discovering of the pregnancy to get an
initial status,
the second time during the late second or early third
trimester and the third time at the time of delivery
because infection or reactivation of syphilis can occur at
any of these times
and every single one of those times would be an opportunity
to treat the mother and potentially to treat the baby.
13:59
What are the clinical manifestations of congenital syphilis?
Early on, meaning when the baby is born, they may show
such sign as enlargement of the liver and spleen,
hepatosplenomegaly.
14:13
They may show some rash, they may show what looks like
post-date skin,
very dry crinkly skin which actually is a manifestation of
that
sort of great overturn of the skin due to prior infection.
14:28
Eventually, as they grow older, they’ll develop these
additional findings several
what you see on the images here.
14:35
Facial abnormalities such a rhagades, yes, that’s pronounced
rha-gades not rha-ge-des,
although some might try to say that in your presence.
14:45
The rhagades are linear scars at the angle of the mouth,
you may think of them as sort of a fan-shape or like what
some people might call angular cheilosis
but the rhagades are due to syphilis occurring in utero.
14:59
That baby’s picture also demonstrates a nasal discharge
and a classic finding in congenital syphilis both early like
in the first couple of days to weeks after delivery
and even later is what it’s called the snuffles, not the
sniffles
such that you get with the common cold but the snuffles.
15:18
Copious, mucopurulent discharge sometimes in fact frequently
progressing
to become overtly bloody, this is something that you could
not miss - it’s very prominent.
15:31
Patients even later in life then, so not in the early
infancy stages
but later still to develop into childhood, adolescence and
adulthood,
may develop what’s called as a saddle nose.
15:43
A saddle nose is best seen in profile the patient.
15:47
I’ll just turn this way for a second.
15:49
The nose itself dips in like that and it becomes sort of a
suppressed saddle shape,
it’s really quite prominent and you can actually see many
examples
of that in historic paintings in the traditional series.
16:02
Teeth, the notched or Hutchinson’s teeth such as you see in
the image on the lower part of the screen,
you can see at almost all of the incisors and even some of
the canines that there’s a central divot if you will,
that would be called Hutchinson’s teeth.
16:20
If you look at the molars in somebody with congenital
syphilis,
they may have what’s called a mulberry molar, this is a fat,
squat,
it looks like a mulberry if you even know what that is,
but it’s a very bulbous looking molar far more so then the
normal smooth walled straight
and purposeful looking molar. Also the maxilla itself may be
shortened.
16:43
Saber shins, certainly something one can see on imaging
as well as looking at the patient, they sort of bow outward and
then cranial nerve VIII deafness.
16:52
So, congenital syphilis is a very bad player when it occurs
and it is so preventable if only we can remember to test and
treat the mothers
at every stage in their pregnancy.