Makindo Medical Notes

Related Subjects: |Treponema |Bejel (Endemic syphilis) |Pinta |Yaws (Frambesia) |Syphilis

Syphilis is thought to have arrived in Barcelona in 1493 from South America, reaching Naples by 1494. Over time, it became recognized as a sexually transmitted disease (venereal disease). Similar spirochetal infections exist in South America. Notable historical figures affected by syphilis include Henry VIII, Ivan the Terrible, and prominent church figures. It led to social changes such as replacing public kissing with handshakes and the adoption of wigs to cover hair loss. The disease was named "Syphilis" after a shepherd in a 1530 poem, and it is also known as "Luetic disease." It has been called by various names throughout Europe, including the "French disease," the "Neapolitan disease," and the "Christian disease" in Turkey. (Reference: Europe - A History, 1997, by Norman Davies)

About

• "To know syphilis is to know medicine." – William Osler
• Syphilis is an infectious sexually transmitted disease caused by Treponema pallidum, a spirochete bacterium.
• Treponema pallidum is a spiral-shaped, corkscrew-like bacterium that cannot be grown in culture.
• The disease possibly originated in South America and spread to Europe in the 15th century.
• Vertical transmission (from mother to baby) can also occur, leading to congenital syphilis.
• Recently, cases have increased in the UK, possibly related to the sex industry and prostitution.
• Always screen for HIV and other sexually transmitted infections (STIs) when syphilis is suspected.

Clinical

• Primary Syphilis: Begins with a painless ulcer (chancre) at the site of inoculation, often the genitals, lips, or anus. The chancre develops from a papule and may heal on its own within a few weeks.
• Secondary Syphilis: Occurs 2-6 weeks later, characterized by systemic symptoms such as malaise, sore throat, and lymphadenopathy. A widespread maculopapular rash may develop, often involving the palms and soles. Other features include condylomata lata (moist, wart-like lesions in the genital area) and nail track ulcers.

Tertiary Syphilis (After 10+ Years)

• CNS Involvement:
  • Syphilitic Meningitis: Can occur during secondary syphilis. Symptoms include headache, neck stiffness, and elevated white cell counts in cerebrospinal fluid (CSF).
  • Meningovascular Syphilis: Occurs 10-20 years after infection, leading to brain and spinal cord infarcts, seizures, cranial nerve lesions, and Argyll Robertson pupils.
  • Tabes Dorsalis: Affects posterior nerve roots, leading to lightning pains, impaired vibration sense, positive Romberg's sign, and Argyll Robertson pupils.
  • General Paresis: Causes progressive dementia, personality changes, and frontal lobe dysfunction. Subdural hematomas can occur in later stages.
  • Gummatous lesions in the brain or spinal cord are rare but possible.
• Cardiovascular Syphilis:
  • Aortic Involvement: Endarteritis of the vasa vasorum can lead to aortitis, aortic regurgitation, and aneurysms.
• Gummas: These soft, tumour-like growths can occur in the skin, liver, bones, and other organs during tertiary syphilis.

Congenital Syphilis

• Increases the risk of late abortion, stillbirth, or neonatal death as Treponema pallidum can cross the placenta.
• Fetal infection may occur if the mother has had untreated syphilis for over five years.
• Clinical features may appear 2-6 weeks after birth, including "snuffles" (nasal discharge) and hepatosplenomegaly.
• Classic signs include Moon's molars and Hutchinson's incisors (notched teeth).
• Bone deformities such as saber shins, osteochondritis, and periostitis are common.
• Other features include saddle nose, interstitial keratitis, and hearing loss due to involvement of the eighth cranial nerve.

Investigations

• Dark Ground Microscopy: Fluid from the lesions can show the presence of spirochetes.
• T. pallidum Enzyme Immunoassay (EIA): The screening test of choice. If positive, it is confirmed by the Treponema Pallidum Hemagglutination Assay (TPHA) and Venereal Disease Research Laboratory (VDRL) test.
• TPHA, fluorescent treponemal antibody (FTA), and EIA remain positive after treatment and can also be positive in cases of yaws (a similar infection).
• VDRL becomes positive after 3 weeks and typically becomes negative after treatment, except in neurosyphilis cases where it may remain positive.
• In cases of suspected neurosyphilis, lumbar puncture may show elevated CSF white cells and protein.
• Screen for other sexually transmitted infections, particularly HIV.

Management

• Primary/Secondary Syphilis: Treatment involves daily intramuscular (IM) injections of procaine benzylpenicillin (2.4 million units) for 10 days or doxycycline 100 mg twice daily for 14 days.
• Tertiary Syphilis: Requires extended treatment with daily IM procaine benzylpenicillin for one month.
• Jarisch-Herxheimer Reaction: This reaction, seen after treatment, is caused by the rapid destruction of spirochetes and is mediated by TNF-alpha, IL-6, and IL-8. It can cause fever, chills, and worsening symptoms but is self-limiting.
• For penicillin-allergic patients, alternative treatments include doxycycline or ceftriaxone.