Tertiary and congenital syphilis. Tertiary and congenital syphilis. Principles of therapy and prophylaxisPrinciples of therapy and prophylaxis..

Lector: Shkilna M. Lector: Shkilna M.

""He who knows syphilis, knows medicine" He who knows syphilis, knows medicine"

Sir William Osler Sir William Osler

ContentContent

1. LATENT SYPHILIS1. LATENT SYPHILIS

2. TERTIARY SYPHILS2. TERTIARY SYPHILS

3. CONGENITAL SYPHILIS3. CONGENITAL SYPHILIS Early congenital syphilis;Early congenital syphilis; Late congenital syphilisLate congenital syphilis

4. Treatment4. Treatment

5. Treatment in Pregnancy5. Treatment in Pregnancy

6. Jarisch-Herxheimer reaction6. Jarisch-Herxheimer reaction

LATENT SYPHILISLATENT SYPHILIS The period between secondary and The period between secondary and

tertiary syphilis. tertiary syphilis. Early latency: the first 4years when secondary Early latency: the first 4years when secondary

relapses may occur.relapses may occur. Late latency is the asymptomatic period Late latency is the asymptomatic period

beyond 4 years. beyond 4 years. During this latter period, the patient During this latter period, the patient

harbors infectious organisms, especially in harbors infectious organisms, especially in the spleen and lymph nodes and blood the spleen and lymph nodes and blood serology remains positive. serology remains positive.

World War II Poster: Both of These Men Had SyphilisWorld War II Poster: Both of These Men Had Syphilis

1/3 of untreated pts will proceed to tertiary syphilis

TERTIARY SYPHILSTERTIARY SYPHILS

is a no contagious but highly destructive phase is a no contagious but highly destructive phase of syphilis which may take many years to of syphilis which may take many years to develop; it may manifest itself in several develop; it may manifest itself in several forms: forms:

GummasGummasNeuro-syphilisNeuro-syphilisCardiovascular SyphilisCardiovascular Syphilis

GummasGummas

It develops in 15% of It develops in 15% of untreated cases within 1-10 untreated cases within 1-10 years after infection.years after infection.

highly destructive tertiary highly destructive tertiary syphilitic lesions that usually syphilitic lesions that usually occur in skin and bones but occur in skin and bones but may also occur in other may also occur in other tissues. tissues.

Slowly progressive, painless, Slowly progressive, painless, dull red nodule or plaque.dull red nodule or plaque.

Breakdown into ulcer with Breakdown into ulcer with wash-leather floor.wash-leather floor.

Regional Ln are not enlarged.Regional Ln are not enlarged. Not infectious.Not infectious.

Late syphilis - ulcerating gummaLate syphilis - ulcerating gumma

Late syphilis - serpiginous gummata Late syphilis - serpiginous gummata of forearmof forearm

Gummas in tertiary syphilis

Cardiovascular Syphilis Cardiovascular Syphilis 10% of untreated syphilis 10% of untreated syphilis

cases develop CDS 10-40 cases develop CDS 10-40 years after initial infection.years after initial infection.

Heart: CDS occurs due to Heart: CDS occurs due to localized affection (gumma) localized affection (gumma) or generalized affection that or generalized affection that leads to heart failure. leads to heart failure.

Aorta: aortic regurge, Aorta: aortic regurge, aneurysm or coronary osteal aneurysm or coronary osteal stenosis.stenosis.

Dilatation of the Aortic Root in Tertiary Syphilis

NeurosyphilsNeurosyphils

AsymptomaticAsymptomaticSymptomaticSymptomatic

Asymptomatic neurosyphilisAsymptomatic neurosyphilis in which there are no symptoms of in which there are no symptoms of

CNS involvement but the CSF is CNS involvement but the CSF is abnormal: abnormal: Elevated lymphocytes Elevated lymphocytes Elevated protein. Elevated protein. Positive CSF VDRL tests Positive CSF VDRL tests Approximately 20% of these patients Approximately 20% of these patients

progress to symptomatic neurosyphilis progress to symptomatic neurosyphilis

Symptomatic Neurosyphilis Symptomatic Neurosyphilis Symptoms of neurosyphilis:Symptoms of neurosyphilis:

8% of untreated cases.8% of untreated cases.5-35 years after infection.5-35 years after infection.

Invasion of the CNS occurs early Invasion of the CNS occurs early when generalized dissemination when generalized dissemination occurs (2° syphilis).occurs (2° syphilis).

Diagnosis of congenital Diagnosis of congenital syphilissyphilis

Mothers anamnesisMothers anamnesis Examination of placenta and umbilical cordExamination of placenta and umbilical cord Assessment of typical characteristics of Assessment of typical characteristics of

congenital syphilis in different growing groupcongenital syphilis in different growing group Treponema pallidum can be demonstrated Treponema pallidum can be demonstrated

in a smear from skin lesions with moist in a smear from skin lesions with moist surface.surface.

Assessment of eye groundAssessment of eye ground An X-ray examination of the long bonesAn X-ray examination of the long bones Clinical assessment of parents.Clinical assessment of parents.

Possible results of pregnancy, Possible results of pregnancy, according to the time of affection according to the time of affection

of pregnant:of pregnant:

Abortion Abortion ( pregnant affected by syphilis before pregnancy or ( pregnant affected by syphilis before pregnancy or during first month of pregnancy)during first month of pregnancy)

Stillbirth Stillbirth ( pregnant affected by syphilis during 4 -5 month of ( pregnant affected by syphilis during 4 -5 month of pregnancy)pregnancy)

Birth of child with Birth of child with congenital syphiliscongenital syphilis ( pregnant affected by ( pregnant affected by syphilis during 6 -7 month of pregnancy)syphilis during 6 -7 month of pregnancy)

If pregnant affected by syphilis during last 1,5-2 month of If pregnant affected by syphilis during last 1,5-2 month of pregnancy, pregnancy, fetus can affected from mother in deliveryfetus can affected from mother in delivery during passing through an infected canalduring passing through an infected canal

CONGENITAL SYPHILISCONGENITAL SYPHILIS

Early congenital syphilis:Early congenital syphilis:occur before the age of 1 year;occur before the age of 1 year;occur in children from 1 to 4 year.occur in children from 1 to 4 year.

a) late syphilitic ophtalmopathy (involvement of a) late syphilitic ophtalmopathy (involvement of the eyes);the eyes);

Late congenital syphilis:Late congenital syphilis:b) other forms of the late congenital syphilis b) other forms of the late congenital syphilis

(involvement of the skin, mucous membrane, (involvement of the skin, mucous membrane, nervous system, latent syphilis).nervous system, latent syphilis).

Syphilis of placentaSyphilis of placenta

PlacentaPlacenta is edematous, is edematous, pale, greasy and bulky.pale, greasy and bulky.

Weight of placentaWeight of placenta is 800-is 800-900 g (500g in normal).900 g (500g in normal).

PlacentaPlacenta is more than one-is more than one-fourth of fetal body weight fourth of fetal body weight

( more than one- fifth in ( more than one- fifth in normal).normal).

Treponema pallidum can Treponema pallidum can be demonstrated in a be demonstrated in a smear fromsmear from placenta placenta

( that’s why it is very ( that’s why it is very contagious).contagious).

Syphilis of fetusSyphilis of fetus

Maceration of fetus.Maceration of fetus. Sometimes dead.Sometimes dead. Little weight.Little weight. Enlargement of liver and spleen.Enlargement of liver and spleen. Lungs are enlarged, thick, grey-yellow in their colorLungs are enlarged, thick, grey-yellow in their color (“Pneumonia Alba”).(“Pneumonia Alba”).

Mucous patches and skin Mucous patches and skin lesions in an infantlesions in an infant

Congenital syphilis:Congenital syphilis:of children before the age of 1 yearof children before the age of 1 year

Syphilitic pemphigusSyphilitic pemphigus Diffuse infiltrationDiffuse infiltration Syphilitic rhinitisSyphilitic rhinitis OsteochondritisOsteochondritis Syphilitic chorioretinitisSyphilitic chorioretinitis

Syphilitic pemphigusSyphilitic pemphigus

Present after burning or appeared Present after burning or appeared within the first days ore weeks of within the first days ore weeks of life.life.

Consists of bullas Consists of bullas

0,5-1 sm.0,5-1 sm. in dm.in dm. Vesicles and erosions distributed Vesicles and erosions distributed

bilaterally and symmetrically on bilaterally and symmetrically on the front of the palms and soles.the front of the palms and soles.

Treponema pallidum can be Treponema pallidum can be demonstrated in a smear from demonstrated in a smear from bulla.bulla.

Diffuse infiltrationDiffuse infiltration Appeared within the first Appeared within the first

weeks or month of birth weeks or month of birth around angles of mouth, around angles of mouth, lips, buttocks, palms, soles lips, buttocks, palms, soles and anus.and anus.

Zones of affection are Zones of affection are flat ,thick ,yellow-brawn in flat ,thick ,yellow-brawn in color with shinny surface.color with shinny surface.

Later fissuring of lips, deep Later fissuring of lips, deep fissures at the angles of the fissures at the angles of the mouth may be seen, after mouth may be seen, after recovering of which we can recovering of which we can see specific fusiform radial see specific fusiform radial atrophic scars.atrophic scars.

Syphilitic rhinitisSyphilitic rhinitis

Nasal breathing is Nasal breathing is difficult.difficult.

Highly infectious Highly infectious purulent-serous and purulent-serous and hemorrhagic nasal hemorrhagic nasal discharges ( snuffles).discharges ( snuffles).

Breathing is hoarse and by Breathing is hoarse and by mouth.mouth.

Nasal septum is dislocated Nasal septum is dislocated or destroyed with the or destroyed with the future deformation of the future deformation of the nose.nose.

Syphilitic chorioretinitisSyphilitic chorioretinitis

Assessment of eye Assessment of eye ground show points of ground show points of pigment and zones of pigment and zones of depigmentations -depigmentations -

symptom of symptom of

““salt- and-peppersalt- and-pepper””

OsteochondritisOsteochondritis

The main specific and The main specific and characteristic changes of the characteristic changes of the bones in patient with congenital bones in patient with congenital syphilis in children before the age syphilis in children before the age of 1 yearof 1 year

Appeared in intrauterine period Appeared in intrauterine period and cant be diagnose till the end and cant be diagnose till the end of 1 year of birthof 1 year of birth

During X-ray examination we can During X-ray examination we can see light strips 0,5-1,5 mm see light strips 0,5-1,5 mm ( breach of ossification cartilage) ( breach of ossification cartilage) between metaphysic and between metaphysic and epiphysisepiphysis

On josses places bones fractures On josses places bones fractures can appearcan appear

Congenital syphilisCongenital syphilis

Papulosquamous Plaques

Congenital syphilis –

snuffles and rash

Rhinitis (snuffles), mucous patches, damage to palate (late) Bullae and vesicular rash on soles

Eroded papular lesions

Broken vesicles, desquamation

Condylomata around anus Infiltration, desquamation and rhinitis

Congenital syphilis:Congenital syphilis:of children from1 to 4 yearof children from1 to 4 year

Condiloma lata can also appeared on genital organs, inguinal folds, perianal zones with Condiloma lata can also appeared on genital organs, inguinal folds, perianal zones with moister surface and large amount of Treponema pallidum .moister surface and large amount of Treponema pallidum .

Pustules, nodules, erosions of mucous membranes of oral cavity.Pustules, nodules, erosions of mucous membranes of oral cavity. Affection of bones, inner organs and nerves system can be present.Affection of bones, inner organs and nerves system can be present. Certain amount of papules in extremities, buttocks, genital organs, on the inguinal folds. They Certain amount of papules in extremities, buttocks, genital organs, on the inguinal folds. They

can be moister. Erosions with a large amount of Treponema pallidum on their surface.can be moister. Erosions with a large amount of Treponema pallidum on their surface.

Late congenital syphilisLate congenital syphilis

Can appear in 5-20 years old and later is Can appear in 5-20 years old and later is characterized by many specific changes in the:characterized by many specific changes in the:

SkinSkinBonesBonesMucous membranesMucous membranesInner organsInner organsNerve systemNerve systemSensitive organsSensitive organs

International classification of International classification of late congenital syphilislate congenital syphilis

Late syphilitic Late syphilitic ophtalmopathic (involvement ophtalmopathic (involvement of the eyes).of the eyes).

Late congenital neurosyphilis.Late congenital neurosyphilis. Others form of late congenital Others form of late congenital

syphilissyphilis

1.1. Affection of bonesAffection of bones

2.2. Affection of teeth Affection of teeth (Hutchinson(Hutchinson’’s teeth)s teeth)

3.3. HutchinsonHutchinson’’s triad s triad ( interstitial keratitis, ( interstitial keratitis, HutchinsonHutchinson’’s teeth, eight s teeth, eight nerve deafness).nerve deafness).

Hutchinson's teethHutchinson's teeth

Characteristic notched edges Characteristic notched edges "screwdriver" shaped central incisors "screwdriver" shaped central incisors

Moon's molar of congenital Moon's molar of congenital syphilissyphilis

Sabre shins (resulting from Sabre shins (resulting from osteoperiosititis of the tibia)osteoperiosititis of the tibia)

Primary, Secondary, Early LatentPrimary, Secondary, Early LatentSyphilisSyphilis

Recommended regimenRecommended regimen-Benzathine Penicillin G, 2.4 million -Benzathine Penicillin G, 2.4 million

units IMunits IM

Penicillin AllergyPenicillin Allergy**-Doxycycline 100 mg twice daily x 14 -Doxycycline 100 mg twice daily x 14

daysdays or or

-Ceftriaxone 1 gm IM/IV daily x 8-10 -Ceftriaxone 1 gm IM/IV daily x 8-10 days (limited studies) days (limited studies)

or or-Azithromycin 2 gm single oral dose -Azithromycin 2 gm single oral dose

(preliminary data)(preliminary data)

SyphilisSyphilisLatent SyphilisLatent Syphilis

Recommended regimenRecommended regimenBenzathine penicillin G 2.4 million units Benzathine penicillin G 2.4 million units IM at one week intervals x 3 dosesIM at one week intervals x 3 doses

Penicillin allergy*Penicillin allergy*Doxycycline 100 mg orally twice daily x 28 daysDoxycycline 100 mg orally twice daily x 28 days

ororTetracycline 500 mg orally four times daily x Tetracycline 500 mg orally four times daily x 28d28d

Latent SyphilisLatent SyphilisResponse to TreatmentResponse to Treatment

Limited data available to guide evaluationLimited data available to guide evaluation

Repeat RPR(VDRL) at 6, 12, 24 monthsRepeat RPR(VDRL) at 6, 12, 24 months

Perform CSF exam & re-treat for latent syphilis, if: Perform CSF exam & re-treat for latent syphilis, if:

4x increase in titer,4x increase in titer,

initial nontreponemal titerinitial nontreponemal titer > >1:32 fails to decline 12-1:32 fails to decline 12-24 mo after tx, or24 mo after tx, or

signs/sxsigns/sx

Jarisch-Herxheimer reactionJarisch-Herxheimer reaction

ManifestationsManifestations: general malaise, fever, headache, sweating, : general malaise, fever, headache, sweating, rigors, or a temporary exacerbation of the syphilitic lesions.rigors, or a temporary exacerbation of the syphilitic lesions.

Usually seen w early syphilis, especially secondary syphilis.Usually seen w early syphilis, especially secondary syphilis. Seen within 6 to 12 h of initial treatment. Seen within 6 to 12 h of initial treatment. Usually subsides within 24 h and poses no danger but may Usually subsides within 24 h and poses no danger but may

produce anxiety. produce anxiety. However, patients with general paresis or a high CSF cell count However, patients with general paresis or a high CSF cell count

are likely to develop serious disorders, such as seizures or are likely to develop serious disorders, such as seizures or strokes. strokes.

It may be confused with allergy to antibiotics.It may be confused with allergy to antibiotics. May indicate coexistent syphilis in patients treated for other May indicate coexistent syphilis in patients treated for other

conditions with antibiotics active against syphilisconditions with antibiotics active against syphilis This reaction should be explained to the patient before treatment.This reaction should be explained to the patient before treatment.