Syphilis

Prof. Ashraf Al-Sawy

Definition
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Syphilis is an infectious disease caused by the spirochete Treponema pallidum. It is almost always transmitted by sexual contact with infectious lesions. But can be transmitted in utero and via blood transfusion.

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Can mimic many other infections and immune-mediated processes in advanced stages "The physician who knows syphilis knows medicine." Sir William Osler

Origin Of Syphilis
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Europe disease: Venetian, Naples, or French disease. New world origin, and holds that sailors who accompanied Columbus and other explorers brought the disease back to Europe. Old world but was not identified as a separate disease from leprosy before about A.D. 1500. Developed in both hemispheres from the related diseases bejel and yaws.

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Clinical Presentation
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Primary

Syphilis
Acquired Congenital

Secondary

Tertiary

Clinical Presentation
S Y P H I L I S 1ry Stage

2ry Stage Cngenital Syphilis 3ry stage

Treponema Pallidum
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Treponemes are helically coiled, corkscrew-shaped cells, 6 to 15 m long and 0.1 to 0.2 m wide.

Epidemiology
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Man is the only known host and transmission is always by direct sexual contact. The incidence is highest in sexually active people (20-29 year old group). 30% of exposed people contract the disease. 30,000 new cases of 1 and 2 syphilis diagnosed per year and approximately 30,000 new cases of early latent syphilis diagnosed per year.

Pathology and Pathogenesis of Syphilis

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Enterance: minute abrasions, m. m. or via hair follicles. Systemic spread via the blood and lymphatics. The most prominent histologic features are vascular changes caused by endarteritis and periarteritis (perivascular cuffing).

Acquired Syphilis
Primary Stage

Primary Stage
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Incubation period: 10 - 90 days. Extensive multiplication of treponemes at the site of entry produces erythema and induration. Papule eventually progresses to a superficial ulcer with a firm base called a hard chancre. Numerous treponemes are present in this highly contagious, open lesion.

Syphilitic Chancres
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Genital (95%) and extragenital (5%). Highly infectious. Usually single. Indurated. Painless. Edge regular. Floor clean. Heal in 3-6 weeks.

Regional lymphadenopathy
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Firm, discrete, mobile, nonsuppurative and painless without overlying skin changes

It may persist for months, despite healing of the chancre.

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Chancre on Female Genitalia

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Multiple Chancre
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Diagnosis
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Clinical picture. Dark ground examination. STS: +ve in 50% of cases after 2 weeks of infection.

Differential Diagnosis
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1- Chancroid: (Haemophilus Ducreyi)

Soft chancre, painful, multiple, bleed easily, L.N. painful and may suppurate. Short IP (2-5 days). Ulcer transient, rapidly disappear LN enlarged, painful, matted may fistulate

2- LGV: (Chlamydia trachomatis)

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3- Genital herpes:

Recurrent, shallow and painful ulcers. LN: may enlarged and tender. Traumatic ulcer, tumors, pyogenic ulcers.

4- Other ulcers:

Secondary Syphilis
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Begin 6-8 weeks after the appearance of the initial chancre May overlap the time when the chancre is still present. The principal manifestations of 2 syphilis are skin and mucous membrane lesions, as well as manifestations of systemic disease.

Secondary Stage
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After an asymptomatic period of 2 to 24 weeks, the secondary or disseminated stage begins Organisms multiply in many different tissues Clinical manifestations include slight fever, generalized lymphadenopathy, malaise, and a mucocutaneous rash

Skin Rash
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Macular Papular Follicular Papulosquamous Pustular.

Round Discrete Nonpruritic Symmetric on the trunk and proximal extremities.

Macular
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Papular
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Maculo-papular
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Papulo-squamous
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Pustular
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M.M. Lesions
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Mucous patches: moist, flat, confluent plaques on mouth, vagina, or anus Painless superficial mucosal erosions, that may develop on the tongue, oral mucosa, lips, vulva, vagina and penis.

Mouth
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Condylomata lata:

wart-like lesions in moist intertriginous areas. Sessile dont bleed easily. D.D.: Condyloma accuminata:

Pedunculated Bleed easily.

All of these lesions teem with treponemes and are highly contagious.

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Anus

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Naso-Labila fold

Generalized lymphadenopathy
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Discrete. Rubbery. Not tender.

Systemic Manifestations:
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Malaise Anorexia Headache Sore throat Arthralgia Low grade fever Nephrotic syndrome.

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2-6 weeks after the onset of secondary syphilis, host defenses bring about healing. About 25 % of untreated patients experience recurrences of this secondary stage in the first several years following infection.

Less Common
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Retinitis. Patchy alopecia (moth eaten alopecia). Hepatitis. Epidedimyitis.

Diagnosis
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Dark ground examination: +ve from m.m. batches & conyloma lata.

Serological tests: +ve in 100% of cases.

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LATENT SYPHILIS

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The period between secondary and tertiary syphilis.

Early latency: the first 4years when secondary relapses may occur. Late latency is the asymptomatic period beyond 4 years.

During this latter period, the patient harbors infectious organisms, especially in the spleen and lymph nodes and blood serology remains positive.

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a positive serological test for syphilis in the absence of any clinical disease symptoms Approximately 25% of patients experience a relapse of 2 syphilis. Only about 1/3 of latent cases progress to 3 syphilis.

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TERTIARY SYPHILS

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is a noncontagious but highly destructive phase of syphilis which may take many years to develop; it may manifest itself in several forms:

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Gummas Neuro-syphilis Cardiovascular Syphils

Gummas
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It develops in 15% of untreated cases within 1-10 years after infection. highly destructive tertiary syphilitic lesions that usually occur in skin and bones but may also occur in other tissues.

Gummas
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Slowely progressive, painless, dull red nodule or plaque. Breakdown into ulcer with wash-leather floor. Regional Ln are not enlarged. Not infectious.

Cardiovascular Syphilis
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10% of untreated syphilis cases develop CDS 10-40 years after initial infection.

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

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Aorta: aortic regurge, aneurysm or coronary osteal stenosis.

Cardiovascular Syphilis
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Approximately 80 percent of fatalities are caused by cardiovascular involvement, while most of the remaining 20 percent are from neurologic involvement.

Neurosyphils
Asymptomatic Symptomatic

Asymptomatic neurosyphilis
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in which there are no symptoms of CNS involvement but the CSF is abnormal:

Elevated lymphocytes Elevated protein. Positive CSF VDRL tests Approximately 20% of these patients progress to symptomatic neurosyphilis

Symptomatic Neurosyphilis
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Symptoms of neurosyphilis:

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

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

Forms of Symptomatic Neurosyphilis

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S meningitis. Meningovascular S. Parenchymatous S.

Brain: generalized paresis. Spinal cord: Tabes dorsalis.

Various combinations.

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Gradual intellectual and emotional deterioration. The middle and posterior arteries are involved weakening in one side. Headache, nausea, vomiting. 3rd, 4th, 6th cranial nerves are affected most often.

Congenital Syphilis
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If a woman is pregnant and has symptomatic or asymptomatic early syphilis, hematogenously disseminating organisms may pass through the placenta to infect the fetus.

Outcome of pregnancy
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1- Abortion. 2- Stillbirth. 3- Child born with signs of syphilis. 4- Born well and then develop early and late congenital syphilis. 5- May escape.

Early Congenital Syphilis

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Signs are apparent before the age of two years These include:

Senile facies and marasmus. Skin rashes: macular, papular or bullous. m.m. lesions: syphilitic rhinitis. Conyloma lata.

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osteochondritis (especially within the long bones) Anemia. Hepatosplenomegaly . Iritis. Meningitis. Death in first 2 years is due to pulmonary hemorrhage, 2 bacterial infection or hepatitis.

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Late Congenital Syphilis

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Syphilitic manifestations appears after 2 years of age.

Manifestations are similar to tertiary syphilis.

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Hutchinson's triad:

interstitial keratitis, Hutchinsons teeth :notched incisors, and eighth-nerve deafness.

Rhagades (radiating fissures at mucocutaneous junctions)

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Neurosyphilis Cardiovascular lesions Clutton's joints (fluid accumulation on knee) Bone deformation of the legs, nasal septum, and hard palate

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Mucous patches and skin lesions in an infant

Hutchinson's teeth
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Characteristic notched edges "screwdriver" shaped central incisors

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Moon's molar of congenital syphilis

Bone abnormalities
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Saddle nose
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Sabre shins (resulting from osteoperiosititis of the tibia)

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Perforation of the hard palate (resulting from gummatous destruction)

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Rhagades facial disfigurement (resulting from persistent syphilitic rhinitis of infancy)

Clutton's joints
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Diagnosis of Syphilis
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1- Dark ground test

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Darkfield examination of exudative material in wet syphilitic lesions or lymph nodes if the lesion is inaccessible (e.g. intraurethral).

2- Serological Tests
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A- Standard tests for syphilis (STS):

Non-specific, so false +ve reactions occurs. Wasserman reaction (WR), VDRL, RPR (rapid plasma reagin).

B- Specific tests: TPI, TPHT, FTA.

3- CSF examination
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If neurosyphilis is suspected.

4- Biopsy
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To differentiate between gumma and tumors.

Therapy of syphilis
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Penicillin is the drug of choice. Benzathine penicillin G but cannot pass the blood brain barrier. Procaine penicillin can pass the blood brain barrier, so protect CNS.

Early Syphilis
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Procaine penicillin 600,000 units daily IM for 10 days or, Benzathine penicillin G 2.4 million units IM in a single dose.

Late Syphilis
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Procaine penicillin 600,000 units daily IM for 20 days or, Benzathine penicillin G 2.4 million units IM every week for 5 weeks (12 million units).

Patients allergic to penicillin

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Tetracycline 500 mg /6hrs for 15 days in early and 30 days in late syphilis.

Erythromycin 500 mg /6hrs for 15 days in early and 30 days in late syphilis.

Congenital Syphilis
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Early S.: Benzathine penicillin G 50,000 units/kg IM, up to the adult dose of 2.4 million units in a single dose. Late S. as late acquired syphilis.

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REACTIONS TO THE TREATMENT

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1- Reactions to penicillin: Anaphylactic shock or urticaria. 2- Therapuetic paradox:

Healing of syphilitic lesions by fibrosis, so the condition becomes more worse (healing of syphilitic aortitis by fibrosis leads to coronary stenosis).

3- Jarisch-Herxheimer Reaction
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Intensification of existing syphilitic lesions and/or exacerbation of old ones following administration of penicillin due to immunological reaction to killed trponemes. The reaction subsides in 24 hours and you should simply warn the patient to expect it.

Prevention
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