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Presenter: Dr Rohini Soni
Moderator: Dr Paschal D’souza
 Syphilis is an infectious
disease caused by the
spirochaetal bacterium
Treponema pallidum subsp.
pallidum.
 Usually acquired through
sexual contact, with the
exception of congenital
syphilis.
 Untreated syphilis infection
can evolve through several
stages, each seperated by a
latency period.
 T. Pallidum is a thin,delicate
,pale motile spirochete.
 Shows to and fro corkscrew
like movement
 Can be visualised with dark
ground microscopy .
 Outer membrane contains
lesser protein content which
helps the organism evades
host immune response
during infection.
TRENDS IN DIAGNOSIS OF SYPHILIS
PRIMARY SYPHILIS
SECONDARY SYPHILIS
NEUROSYPHILIS CARDIOVASCULAR LATE BENIGN
 T. pallidum is presumed to penetrate the skin
through small breaks in the skin or mucosa. At
this site there forms a primary chancre.
 Here they multiply and body tries to eliminate
them through cellular and humoral response
.primary chanchre involutes but there comes a
stage of spirochetemia.
 A lot of antigen antibody complexes a formed
which may manifestation of secondary syphilis.
 After that there is a latency period when no
skin rash is there .
 At this stage this AgAb complexes as well as
few slow growing treponema enters the
various organs such as brain ,eyes ,liver,heart
etc to produce systemic manifestation
 One of the most dreaded complication of
syphilis.
 Two forms:
1. Early : associated with CSF changes and
involvement of cerebral blood vessels and
meninges.
2. Late: mainly involve meninges , brain and
spinal cord parenchyma.
Treponema invades the CNS
Asymtomatic syphilitic
meningitis
CSF abnormalities
with no symptoms
Resolves
spontaneously
Meningovascular syphilis
If left untreated
Infiltrate of lymphocytes and plasma cells
present around blood vessels and prolonged
inflammation leads to fibroblastic changes
Obstruction to CSF and
hydrocephalus Compression with exudates
Cranial nerve palsy
Endarteritis of cerebral
Arteries causes
Cerebral infarction
 There are no clinical manifestations of asymptomatic
neurosyphilis .
 Defined by the presence of abnormalities in the CSF in
the absence of other findings of neurologic disease.
 The usual abnormalities include
1. 10–100 WBC/mm3 (nearly all of which are
lymphocytes)
2. protein of 50–100 mg/dL
3. a reactive nontreponemal antibody (Venereal
Disease Research Laboratory [VDRL]) test in the
CSF.
4. Blood serology (VDRL or rapid plasma reagin [RPR])
is usually but not invariably positive
 The incubation period in the majority of patients
with syphilitic meningitis is less than 1 year.
 Symptoms include acute or subacute onset of
headache, neck stiffness, nausea or vomitting
with signs of increased ICP.
 Basilar meningitis may involve multiple crainial
nerves.
 Sensorineural deafness may also be seen in few
cases.
 Vascular neurosyphilis may involve any part of
the central nervous system.
 The common denominator is infarction
secondary to syphilitic endarteritis.
 The middle cerebral artery, commonest to be
included but any other artery on occasion may
be occluded.
 Presents with hemiparesis or hemiplegia (83% of
cases), aphasia (31%), and seizures (14%).
 Angiographic changes include diffuse irregularity and
“beading” of anterior and middle cerebral arteries and
segmental dilatation of the pericallosal artery.
 Serum RPR is positive in meningovascular syphilis.
 Computed tomography (CT) shows low density areas
with variable degrees of contrast enhancement,
consistent with multifocal infarctions
 CSF findings are positive.
 Meningovascular syphilis of the spinal cord consists
principally of syphilitic meningomyelitis (the most
common form) and spinal vascular syphilis.
 Rare complication
 weakness or paresthesias of the legs, progressing to
paraparesis or paraplegia, which is often asymmetric.
 Urinary and fecal incontinence and variable sensory
disorders (pain and paresthesias) .
 On examination, the legs are weak and spastic, and deep
tendon reflexes are hyperactive; ankle clonus is present.
 Characterised by dementia ,memory
impairment ,disordered judgement and
disturbed affect- manic behaviour ,delusion
of grandeur etc.
 Two phases:
1. Pre paralytic: with progressive dementia
2. Paralytic: corticospinal and extrapyrimidal
symptoms and signs develop a/w
involuntary movements.
 Argyll Robertson type of pupil may develop
 Flattening of the facial lines, tremors of the lips,
tongue, facial muscles, and fingers; and impaired
handwriting and speech.
 Communicating hydrocephalus may complicate the
case.
 CSF may be normal. Specific antitreponemal
antibody tests of CSF, such as FTA-ABS or micro-
hemagglutination assay for T. pallidum MHA-TP.
 CSF-IgG index
 Grossly, the brain in general paresis shows
varying degrees of thickening of the meninges,
consistent with chronic meningitis and fibrosis.
 Cerebral atrophy is prominent, particularly in the
frontal pole and the tips of the temporal lobes.
 Demyelination of cerebral white matter is often
present.
 Late manifestation of untreated tertiary
syphilis.
 Locomotor ataxia
 There is slow degeneration of myelinated
sensory nerve fibres specially present in
dorsal column of the spinal cord
Lightning pains
Bladder
disturbances
Sensory ataxia
 The eye can be impacted by syphilis at any
stage of the disease.
 The ability of syphilis to mimic different
ocular disorders can lead to misdiagnosis and
delay in appropriate antimicrobial therapy.
 Uveitis was the most common ocular
manifestation seen.
 Uveitis, which may be unilateral or
bilateral, is one of the most
frequent ocular manifestations of
syphilis.
 Granulomatous features, including
large keratic precipitates (and iris
nodules.
 Dilated iris vessels, known as iris
roseolae occur rarely, but are
relatively specific for syphilis.
 Along with all forms of herpes virus
infection, sarcoidosis and
toxoplasmosis, syphilis is a common
cause of elevated intraocular
pressure associated with uveitis, or
so-called Inflammatory Ocular
Hypertension Syndrome
 Common long-term complications of
syphilitic uveitis include glaucoma, cataract,
epiretinal membrane and macular edema.
 Choroidal neovascularization, while
uncommon, has been described and may
resolve following treatment with penicillin.
 Syphilitic optic atrophy, which often is seen in tabes,
may appear as an isolated manifestation of
neurosyphilis.
 The usual symptoms of optic atrophy are those of
progressive visual loss involving first one eye and then
the other.
 CSF abnormalities are usually present in the untreated
patient.
 Penicillin treatment can usually prevent further
progression of visual loss.
 Otosyphilis is well known as a complication of syphilis.
 usually diagnosed by various cochleo-vestibular
symptoms, a positive serological test (TPHA, RPR and or
VDRL) and the exclusion of other causes.
 As a part of neurosyphilis , it can involve 8 th nerve usually
effecting hearing as well as balance
 Accompanied sensineuronal hearing loss may be unilateral
or bilateral, usually progresses rapidly and may have a
sudden onset .
 In addition to intravenous antibiotics, corticosteroids are
usually administered in otosyphilis.
 Bones may be affected at all stages of syphilis,
but is more characteristic of tertiary syphilis and
congenital infection.
 Bone lesions in patients with secondary syphilis
most often involve the skull and the long bones
of the limbs, particularly the lower limbs.
 The most common skeletal lesion in early
syphilis is periostitis, most frequently affecting
the tibiae, followed by the skull, sternum and
ribs.
 Osteolytic lesions are less frequent and most
commonly affect the skull and
sternoclavicular junction.
 Patients may present with both periostitis
and osteolytic lesions.
 Involvement of the skull most commonly
affects the frontal and/or parietal bones
 These lesions start as a small
depression on the skull, called
Caries Sicca, associated with
inflammation in the tissue
immediately in contact with
the bone.
 This gradually deepens and
widens and later new bone is
deposited around the edge,
giving a slight ridge (called a
periosteal reaction).
 There can be similar reactions
on other bones of the body.
 Syphilis of the cardiovascular system becomes clinically
manifest after a latent period of 15–30 years.
 Most patients are between 40 and 55 years of age at
onset, and men are affected three times as often as
women.
 Cardiovascular syphilis may lead to aortic aneurysms,
aortic insufficiency, coronary artery stenosis, and, rarely,
myocarditis.
 Its clinical presentation is characterized by the functional
disorder resulting from cardiac involvement, and, at times,
it may be difficult to distinguish cardiovascular syphilis
from other more common varieties of cardiac disease.
T . pallidum
Lodge in vasa vasorum of
aorta .
Spread via lymphatics
Transmural inflammatory lesion
Obliterative endarteritis
Specially involving
ostium of coronary
artery
Aortic media develops patchy necrosis with focal scarring
Elastic tissue gets destroyed
AORTIC
DILATION &
ANEURYSM
Intima becomes atherosclerotic
Extensive plaque
formation
TREE BARK
APPEARENCE
Calcification of the intima
EGG SHELL
CALCIFICATION
 virtually always involve the thoracic aorta,
particularly the ascending aorta immediately at
and above the sinuses ofValsalva.
 Over 60% involve the ascending portion of the
thoracic aorta.
 These aneurysms are typically fusiform or
saccular in type.
 Mostly asymptomatic
 Symptoms eventually develop when an
aneurysm encroaches on surrounding
structures or ruptures.
 Sometimes can compress RLN.
 Rarely SuperiorVena Cava syndrome may
also develop.
 The coronary arteries may be primarily involved
in syphilis, but almost always only the ostia or
the most proximal few millimeters of the
coronary arteries are affected.
 The pathogenesisis an obliterative endarteritis.
 When the luetic process significantly narrows
the coronary ostia, it may lead to ischemic heart
disease, including angina pectoris or sudden
death.
 Pure aortic regurgitation without stenosis
formerly manifestation of syphilis, occurring in
roughly 30%.
 Aortic regurgitation appears to be due to aortic
root dilation with stretching of the aortic valve,
leading in many cases to widening of the aortic
valve commissures, thickening of the aortic
valve leaflets, and a variable amount of aortic
valve incompetence.
 Late benign syphilis or gumma is a proliferative
granulomatous inflammatory process that may
be destructive of affected tissues.
 Gummas are nodules that may be found in any
tissue or organ.
 Histologic picture shows coagulative necrosis
surrounded by lymphocytes and mononuclear
cells; multinucleated giant cells appear only
rarely.
 The solitary gumma :a subcutaneous process that involves
the skin secondarily. It is more common on the thighs,
buttocks, shoulders, forehead, and scalp. As it becomes
necrotic, it has the characteristics of a “cold abscess,”.
 Skelton: include periostitis, gummatous osteitis, and
sclerosing osteitis.
 GIT: gumma of the liver is a frequent type of
gastrointestinal tertiary syphilis.
 Upper respiratory tract, mouth, and tongue. Gummatous
osteitis of the nasal bones, hard palate, and nasal septum,
as well as perichondritis.
Systemic manifestation of acquired syphilis
Systemic manifestation of acquired syphilis
Systemic manifestation of acquired syphilis
Systemic manifestation of acquired syphilis

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Systemic manifestation of acquired syphilis

  • 1. Presenter: Dr Rohini Soni Moderator: Dr Paschal D’souza
  • 2.
  • 3.  Syphilis is an infectious disease caused by the spirochaetal bacterium Treponema pallidum subsp. pallidum.  Usually acquired through sexual contact, with the exception of congenital syphilis.  Untreated syphilis infection can evolve through several stages, each seperated by a latency period.
  • 4.  T. Pallidum is a thin,delicate ,pale motile spirochete.  Shows to and fro corkscrew like movement  Can be visualised with dark ground microscopy .  Outer membrane contains lesser protein content which helps the organism evades host immune response during infection.
  • 5. TRENDS IN DIAGNOSIS OF SYPHILIS
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  • 13.  T. pallidum is presumed to penetrate the skin through small breaks in the skin or mucosa. At this site there forms a primary chancre.  Here they multiply and body tries to eliminate them through cellular and humoral response .primary chanchre involutes but there comes a stage of spirochetemia.  A lot of antigen antibody complexes a formed which may manifestation of secondary syphilis.
  • 14.  After that there is a latency period when no skin rash is there .  At this stage this AgAb complexes as well as few slow growing treponema enters the various organs such as brain ,eyes ,liver,heart etc to produce systemic manifestation
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  • 17.  One of the most dreaded complication of syphilis.  Two forms: 1. Early : associated with CSF changes and involvement of cerebral blood vessels and meninges. 2. Late: mainly involve meninges , brain and spinal cord parenchyma.
  • 18. Treponema invades the CNS Asymtomatic syphilitic meningitis CSF abnormalities with no symptoms Resolves spontaneously Meningovascular syphilis If left untreated Infiltrate of lymphocytes and plasma cells present around blood vessels and prolonged inflammation leads to fibroblastic changes Obstruction to CSF and hydrocephalus Compression with exudates Cranial nerve palsy Endarteritis of cerebral Arteries causes Cerebral infarction
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  • 20.  There are no clinical manifestations of asymptomatic neurosyphilis .  Defined by the presence of abnormalities in the CSF in the absence of other findings of neurologic disease.  The usual abnormalities include 1. 10–100 WBC/mm3 (nearly all of which are lymphocytes) 2. protein of 50–100 mg/dL 3. a reactive nontreponemal antibody (Venereal Disease Research Laboratory [VDRL]) test in the CSF. 4. Blood serology (VDRL or rapid plasma reagin [RPR]) is usually but not invariably positive
  • 21.  The incubation period in the majority of patients with syphilitic meningitis is less than 1 year.  Symptoms include acute or subacute onset of headache, neck stiffness, nausea or vomitting with signs of increased ICP.  Basilar meningitis may involve multiple crainial nerves.  Sensorineural deafness may also be seen in few cases.
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  • 23.  Vascular neurosyphilis may involve any part of the central nervous system.  The common denominator is infarction secondary to syphilitic endarteritis.  The middle cerebral artery, commonest to be included but any other artery on occasion may be occluded.  Presents with hemiparesis or hemiplegia (83% of cases), aphasia (31%), and seizures (14%).
  • 24.  Angiographic changes include diffuse irregularity and “beading” of anterior and middle cerebral arteries and segmental dilatation of the pericallosal artery.  Serum RPR is positive in meningovascular syphilis.  Computed tomography (CT) shows low density areas with variable degrees of contrast enhancement, consistent with multifocal infarctions  CSF findings are positive.
  • 25.  Meningovascular syphilis of the spinal cord consists principally of syphilitic meningomyelitis (the most common form) and spinal vascular syphilis.  Rare complication  weakness or paresthesias of the legs, progressing to paraparesis or paraplegia, which is often asymmetric.  Urinary and fecal incontinence and variable sensory disorders (pain and paresthesias) .  On examination, the legs are weak and spastic, and deep tendon reflexes are hyperactive; ankle clonus is present.
  • 26.  Characterised by dementia ,memory impairment ,disordered judgement and disturbed affect- manic behaviour ,delusion of grandeur etc.  Two phases: 1. Pre paralytic: with progressive dementia 2. Paralytic: corticospinal and extrapyrimidal symptoms and signs develop a/w involuntary movements.
  • 27.  Argyll Robertson type of pupil may develop  Flattening of the facial lines, tremors of the lips, tongue, facial muscles, and fingers; and impaired handwriting and speech.  Communicating hydrocephalus may complicate the case.  CSF may be normal. Specific antitreponemal antibody tests of CSF, such as FTA-ABS or micro- hemagglutination assay for T. pallidum MHA-TP.  CSF-IgG index
  • 28.  Grossly, the brain in general paresis shows varying degrees of thickening of the meninges, consistent with chronic meningitis and fibrosis.  Cerebral atrophy is prominent, particularly in the frontal pole and the tips of the temporal lobes.  Demyelination of cerebral white matter is often present.
  • 29.  Late manifestation of untreated tertiary syphilis.  Locomotor ataxia  There is slow degeneration of myelinated sensory nerve fibres specially present in dorsal column of the spinal cord
  • 31.  The eye can be impacted by syphilis at any stage of the disease.  The ability of syphilis to mimic different ocular disorders can lead to misdiagnosis and delay in appropriate antimicrobial therapy.  Uveitis was the most common ocular manifestation seen.
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  • 34.  Uveitis, which may be unilateral or bilateral, is one of the most frequent ocular manifestations of syphilis.  Granulomatous features, including large keratic precipitates (and iris nodules.  Dilated iris vessels, known as iris roseolae occur rarely, but are relatively specific for syphilis.  Along with all forms of herpes virus infection, sarcoidosis and toxoplasmosis, syphilis is a common cause of elevated intraocular pressure associated with uveitis, or so-called Inflammatory Ocular Hypertension Syndrome
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  • 37.  Common long-term complications of syphilitic uveitis include glaucoma, cataract, epiretinal membrane and macular edema.  Choroidal neovascularization, while uncommon, has been described and may resolve following treatment with penicillin.
  • 38.  Syphilitic optic atrophy, which often is seen in tabes, may appear as an isolated manifestation of neurosyphilis.  The usual symptoms of optic atrophy are those of progressive visual loss involving first one eye and then the other.  CSF abnormalities are usually present in the untreated patient.  Penicillin treatment can usually prevent further progression of visual loss.
  • 39.  Otosyphilis is well known as a complication of syphilis.  usually diagnosed by various cochleo-vestibular symptoms, a positive serological test (TPHA, RPR and or VDRL) and the exclusion of other causes.  As a part of neurosyphilis , it can involve 8 th nerve usually effecting hearing as well as balance  Accompanied sensineuronal hearing loss may be unilateral or bilateral, usually progresses rapidly and may have a sudden onset .  In addition to intravenous antibiotics, corticosteroids are usually administered in otosyphilis.
  • 40.  Bones may be affected at all stages of syphilis, but is more characteristic of tertiary syphilis and congenital infection.  Bone lesions in patients with secondary syphilis most often involve the skull and the long bones of the limbs, particularly the lower limbs.  The most common skeletal lesion in early syphilis is periostitis, most frequently affecting the tibiae, followed by the skull, sternum and ribs.
  • 41.  Osteolytic lesions are less frequent and most commonly affect the skull and sternoclavicular junction.  Patients may present with both periostitis and osteolytic lesions.  Involvement of the skull most commonly affects the frontal and/or parietal bones
  • 42.  These lesions start as a small depression on the skull, called Caries Sicca, associated with inflammation in the tissue immediately in contact with the bone.  This gradually deepens and widens and later new bone is deposited around the edge, giving a slight ridge (called a periosteal reaction).  There can be similar reactions on other bones of the body.
  • 43.  Syphilis of the cardiovascular system becomes clinically manifest after a latent period of 15–30 years.  Most patients are between 40 and 55 years of age at onset, and men are affected three times as often as women.  Cardiovascular syphilis may lead to aortic aneurysms, aortic insufficiency, coronary artery stenosis, and, rarely, myocarditis.  Its clinical presentation is characterized by the functional disorder resulting from cardiac involvement, and, at times, it may be difficult to distinguish cardiovascular syphilis from other more common varieties of cardiac disease.
  • 44. T . pallidum Lodge in vasa vasorum of aorta . Spread via lymphatics Transmural inflammatory lesion Obliterative endarteritis Specially involving ostium of coronary artery Aortic media develops patchy necrosis with focal scarring
  • 45. Elastic tissue gets destroyed AORTIC DILATION & ANEURYSM Intima becomes atherosclerotic Extensive plaque formation TREE BARK APPEARENCE Calcification of the intima EGG SHELL CALCIFICATION
  • 46.  virtually always involve the thoracic aorta, particularly the ascending aorta immediately at and above the sinuses ofValsalva.  Over 60% involve the ascending portion of the thoracic aorta.  These aneurysms are typically fusiform or saccular in type.  Mostly asymptomatic
  • 47.  Symptoms eventually develop when an aneurysm encroaches on surrounding structures or ruptures.  Sometimes can compress RLN.  Rarely SuperiorVena Cava syndrome may also develop.
  • 48.  The coronary arteries may be primarily involved in syphilis, but almost always only the ostia or the most proximal few millimeters of the coronary arteries are affected.  The pathogenesisis an obliterative endarteritis.  When the luetic process significantly narrows the coronary ostia, it may lead to ischemic heart disease, including angina pectoris or sudden death.
  • 49.  Pure aortic regurgitation without stenosis formerly manifestation of syphilis, occurring in roughly 30%.  Aortic regurgitation appears to be due to aortic root dilation with stretching of the aortic valve, leading in many cases to widening of the aortic valve commissures, thickening of the aortic valve leaflets, and a variable amount of aortic valve incompetence.
  • 50.  Late benign syphilis or gumma is a proliferative granulomatous inflammatory process that may be destructive of affected tissues.  Gummas are nodules that may be found in any tissue or organ.  Histologic picture shows coagulative necrosis surrounded by lymphocytes and mononuclear cells; multinucleated giant cells appear only rarely.
  • 51.  The solitary gumma :a subcutaneous process that involves the skin secondarily. It is more common on the thighs, buttocks, shoulders, forehead, and scalp. As it becomes necrotic, it has the characteristics of a “cold abscess,”.  Skelton: include periostitis, gummatous osteitis, and sclerosing osteitis.  GIT: gumma of the liver is a frequent type of gastrointestinal tertiary syphilis.  Upper respiratory tract, mouth, and tongue. Gummatous osteitis of the nasal bones, hard palate, and nasal septum, as well as perichondritis.