Syphilis

Syphilis is a sexually transmitted disease caused by the spirochete bacterium Treponema pallidum. It is almost always transmitted through sexual contact with infectious lesions, but it also can be transmitted in utero and via blood transfusion. Syphilis is also called “the great imitator” because many of its symptoms are almost indistinguishable from those of other diseases. Other names are: “syph”, “the pox” (or “great pox”), “lues”, and the “French disease”.

The word “syphilis” comes from Greek and it means “lover of swine” (sus-philos) or “one who makes love” (sym-philos). It was the name of the main character in a Latin epic poem written in 1530 by the Italian physician and poet, Girolamo Fracastoro, called Syphilis sive morbus gallicus (the Latin for “Syphilis or The French Disease”). Syphilis was a shepherd who contracted the disease as a punishment from the god Apollo for the defiance Syphilis and his followers showed him.

The incidence of syphilis had been declining in recent years, with 53,000 reported cases (11,387 primary and secondary cases) in 1996, compared with 113,000 cases (33,962 primary and secondary cases) reported in 1992. However, the number of cases of primary and secondary syphilis increased yearly from 2000-2003. In 2003, 7177 cases were reported to the US Centers for Disease Control and Prevention. Most of this increase has been noted in men, particularly in men who have sex with other men. The overall cases reported in women decreased. More than 80% of cases were reported in the southern United States. Trends for congenital syphilis cases closely parallel those for acquired syphilis cases in women, namely, a decreased incidence over the past decade.

Internationally, Syphilis remains prevalent in many developing countries and in some areas of North America, Asia, and Europe, especially Eastern Europe. In some regions of Siberia, as of 1999, prevalence was 1300 cases per 100,000 population.      

SIGNS AND SYMPTOMS 

Syphilis usually has an incubation period of approximately 10 up to 90 days, with an average of 3 weeks. This is when primary Syphilis appears.

Syphilis symptoms occur in stages: primary syphilis, secondary syphilis, latent syphilis, and tertiary syphilis.

Primary syphilis is usually marked by the appearance of painless sores (called chancres) on the penis or scrotum of 70% of men with syphilis and on the vulva, cervix, or perineum of more than 50% of women with syphilis. The chancre usually heals within 4-8 weeks, with or without treatment. The primary lesion has a surrounding red areola. The edge and base of the ulcer have a buttonlike consistency on touching. When abraded, this chancre releases a clear serum containing numerous T pallidum organisms. Extragenital lesions can also occur above the neck, typically affecting the lips or oral cavity. The regional lymph nodes usually enlarge painlessly and are firm, discrete, and nontender. Nevertheless, there are other diseases that can be mistaken for primary syphilitic lesions. These include herpes simplex (primary and recurrent infection), chancroid, traumatic superinfected lesions, carcinoma (a malignant tumor that begins in the lining layer of organs; at least 80% of all cancers are carcinomas), mycotic infection, granuloma inguinale, lichen planus (a common skin disease in which itchy, small, pink or purple spots appear on the arms or legs), psoriasis (a chronic disease of the skin marked by red patches covered with white scales), fungal infection, venereal chlamydial infections.

Secondary syphilis is characterized by the appearance of a cutaneous eruption (skin rash) within 2-10 weeks after the primary chancre and is most florid 3-4 months after infection. The eruption may be subtle (25% of patients may be unaware of skin changes). It can spread over the entire body or it may be limited to certain areas. These lesions are red and have 3-10 mm in diameter. The eruption can be contagious, so it is important to avoid skin-to-skin contact with an uninfected person. The lesions often become necrotic and are distributed widely with frequent involvement of the palms and soles. Other symptoms of secondary syphilis include mild symptoms of malaise, headache, anorexia, nausea, aching pains in the bones, and fatigue often are present, as well as fever and neck stiffness. A small number of patients develop acute syphilitic meningitis and experience headache, neck stiffness, facial numbness or weakness, and deafness. Secondary syphilis symptoms can also disappear without treatment and they can reoccur for up to 2 years before progressing to the next stage of the disease. Drug eruptions, pityriasis rosea (a common skin condition characterized by scaly, pink, and inflamed skin), psoriasis, lichen planus, viral exanthema (a widespread rash) may be mistaken for secondary syphilis due to the high resemblance of the eruption.

Some of the people infected with syphilis may experience a latent (hidden) stage, during which all symptoms disappear. This stage can last many years, but it is still contagious. In the late stages of syphilis, it may subsequently damage the internal organs, including the brain, nerves, eyes, heart, blood vessels, liver, bones, and joints. This internal damage may show up many years later. Symptoms of the late stage of syphilis include difficulty coordinating muscle movements, paralysis, numbness, gradual blindness, and dementia. This damage may be serious enough to cause death.

Tertiary syphilis usually appears within 3-10 years of infection. The typical lesion is a gumma (local lesion with soft tumor-like formations, histiocytes), one can also experience bone pain, which is described as a deep boring pain characteristically worse at night. Gummas may be identified on the skin, in the mouth, and in the upper respiratory tract. They appear most commonly on the leg just below the knee. Gummas may be multiple or diffuse but usually are solitary lesions that range from less than 1 cm to several centimetres in diameter. There can also appear symptoms representative for the area affected, e.g. brain involvement (headache, dizziness, mood disturbance, neck stiffness, blurred vision) and spinal cord involvement (bulbar symptoms, weakness and wasting of shoulder girdle and arm muscles, incontinence, impotence). Some people may experience, 20 years after infection, behavioral changes and other signs of dementia, which is indicative of neurosyphilis.

Congenital syphilis is syphilis present in utero and at birth, and occurs when a child is born to a mother with secondary or tertiary syphilis. The infection inside the uterus mostly occurs during the fifth month. According to the CDC, 40 % of the births to syphilitic mothers are stillborn, 40-70 % of the survivors will be infected, and 12 % of these will die prematurely. The manifestations of untreated congenital syphilis can be divided into those that are expressed prior to age 2 years (early) or after age 2 years (late). The early manifestations include abnormal x-rays (61%), hepatomegaly (enlarged liver) (51%), splenomegaly (enlarged spleen) (49%), petechiae (tiny localized hemorrhages from the small blood vessels just beneath the surface of the skin) (41%), other skin rashes (35%), anemia (34%), lymphadenopathy (swelling of the lymph nodes) (32%), jaundice (30%), pseudoparalysis (28%), and snuffles (obstructed nasal respiration) (23%). Late manifestations are rare and, if encountered, usually involve complications including interstitial keratitis (inflammation of the cornea), cranial nerve VIII deafness, corneal opacities, and/or recurrent arthropathy (joint disease). Dental abnormalities may be evident, such as centrally notched and widely spaced, peg-shaped, upper central incisors (Hutchinson teeth) and sixth-year molars with multiple poorly developed cusps (mulberry molars). Death from congenital syphilis is usually through pulmonary haemorrhage. Affected children are highly infectious until about 2 years old.

Neurosyphilis appears when the infection spreads to the neurological system and it may occur during any stage of syphilis. It may be symptomatic or asymptomatic. The symptomatic neurosyphilis can manifest as syphilitic meningitis (an infection of the lining of the brain), meningovascular syphilis, or parenchymatous neurosyphilis (the parenchyma is the tissue of an organ). Syphilitic meningitis develops within several years of initial infection and the symptoms are the symptoms of meningitis, including headache, nausea and vomiting, and photophobia, but are typically afebrile. Meningovascular syphilis usually manifests 5-10 years after infection and is the result of endarteritis (inflammation of the inner lining of an artery), which affects small blood vessels of the meninges (the three membranes pia mater, arachnoid mater, and dura mater that surround the brain and spinal cord), brain, and spinal cord. Parenchymatous neurosyphilis results from direct parenchymal CNS (Central Nervous System) invasion by T pallidum and is usually a late development (15-20 years after primary infection). Symptoms of parenchymatous reurosyphilis include ataxia (loss of coordination), incontinence (the inability to control urination), paresthesias (abnormal touch sensations, such as burning or prickling, that occur without an outside stimulus), and loss of position, vibratory, pain, and temperature sensations. Paresis and dementia, with changes in personality and intellect, may develop. 

TESTING 

It is very important to get tested if you think you have syphilis or if you have had intimate contact with someone who does.

Syphilis can be easily diagnosed, at any stage, by running several types of tests. These are Rapid Plasma Reagin (RPR), Venereal Disease Research Laboratory (VDRL), Treponema pallidum haemagglutination assay (TPHA) or Fluorescent Treponemal Antibody Absorption (FTA-ABS). There is also another quick and effective test consisting of a Simple microscopy of chancre fluid using dark ground illumination.  

TREATMENT 

 The current treatment for primary, secondary and early latent syphilis is penicillin, in the form of Benzathine penicillin G, 2.4 MU IM in a single dose. For penicillin-allergic persons the treatment consists of a 2-week course of Doxycycline 100 mg PO bid, Tetracycline 500 mg PO qid, or Erythromycin base 500 mg PO qid.

For late latent syphilis (>1 y duration), syphilis of undetermined duration, and late syphilis, the treatment consists of Benzathine penicillin G, 2.4 million U IM once weekly for 3 consecutive weeks or Doxycycline 100 mg PO bid or tetracycline 500 mg PO qid daily for 4 weeks for penicillin-allergic persons.

For neurosyphilis the treatment is aqueous Crystalline Penicillin G, 2-4 million U IV q4h for 10-14 days or Procaine Penicillin, 2.4 million U IM qd, plus Probenecid 500 mg PO qid for 10-14 days.

Syphilis treatment may develop side-effects which are known as the Jarisch-Herxheimer reaction and include transient fever and symptoms such as malaise, chills, headache, and myalgias (muscular pain or tenderness, especially when diffuse and nonspecific), intensification of existing lesions. The reaction is quite common, develops within several hours after beginning antibiotic treatment, and usually clears within 24 hours.

Patients treated for primary and secondary syphilis should have follow-up VDRL at 3, 6, and 12 months after treatment. Those with HIV should be monitored closely as they are known to have more rapid progression of disease. Patients with neurosyphilis should have follow-up at 6-month intervals for at least 3 years with physical examinations and CSF (cerebrospinal fluid) and serologic testing. Pregnant women treated for syphilis should have monthly VDRL testing for the duration of their pregnancy. 

COMPLICATIONS 

Left untreated, syphilis eventually leads to blindness, loss of motor control skills, dementia and death.

Most of the complications appear during the third stage of the disease. During this stage, syphilis can spread to other parts of the body and affect the nerves, heart, brain, eyes, internal organs, joints, liver and bones. Death may result in approximately 20% of untreated patients.

During pregnancy, syphilis can have devastating consequences in the child if left untreated so it is very important to recognize the symptoms and seek medical care. 

PREVENTION 

In order to prevent contracting syphilis you must never have unprotected sex. But if you happen to experience symptoms similar to those of syphilis you should seek medical care as soon as possible because early stages of syphilis can be easily treated.

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