Syphilis is a serious, chronic infectious disease caused by the bacterium Treponema pallidum. It is a sexually-transmissible disease that passes through characteristic stages of signs and symptoms in its natural untreated course. Untreated syphilis is characterized by a lengthy course with periodic remission and exacerbation accompanied by formation of specific local inflammations in all organs and tissues.
A distinction is made between the following syphilis stages:
Incubation period lasts from the moment of infection till appearance of a hard chancre. Length of the period is 3-4 weeks (may vary from 10 to 80 days). The latent period may be longer during admission of antibiotics.
Primary syphilis is characterized by appearance of a hard chancre or primary syphiloma at the location of Treponema pallidum entrance.
Up to 50% of individuals with untreated syphilis may develop tertiary syphilis with lesions many years after the initial infection. The lesions in the tertiary stage may involve the skin, mucous membranes, bones, joints, and irreversibly affected internal organs.
Syphilis occurs most commonly amongst those aged 20 to 24.
The development of syphilis occurs in four stages: primary, secondary, latent and late stage (tertiary).
Primary syphilis is characterized by a chancre, which is a single, painless ulceration with a firm border, that appears at the site of infection. As a rule no inflammation signs can be observed around the chancre. The central part of the hard chancre may look covered with a dense grey-yellowish tarnish. The chancre diameter is 10-20mm.
The chancre usually appears on the external genitalia (glans penis, inner and outer lobes of the foreskin, seldom on the skin of the scrotum and pubis, on the major and minor lips), but can also appear in the anal canal or mouth, on the tongue, lips, nipples or throat (tonsils). It can in fact appear anywhere on the body. It occurs approximately 21 days after contact (although this can range from 10 days to 3 months) and usually heals within six weeks, even without medication. Enlargement of lymph nodes located in the groin, arm pits and neck occurs within one week of appearance of the chancre.
Signs of secondary syphilis begin to appear within six weeks to six months after contact with a rash over all or part of the body. Active bacteria are present in the sores of the rash; these sores are lesions of the skin or mucous membranes. The skin rash that occurs ranges from flat to raised to pustular or blister-like lesions. These lesions characteristically occur on the palms of the hands, soles of the feet, face, and scalp. The raised lesions may break down in moist areas of skin folds to form broad gray-white or pink lesions. The breaking down of mucous membranes, known as mucous patches, appears on lips, inside the mouth, vulva, and vagina. With secondary syphilis there may also be headaches, fever, fatigue, weight loss, sore throat, patchy hair loss, enlarged lymph nodes and loss of appetite. This stage of symptoms will also pass without medication, but may reappear over the next 1 to 2 years. This does not mean the disease is gone – just the symptoms.
Untreated syphilis then goes into a noncontagious latent period – latent syphilis. It occurs when the laboratory tests for syphilis are positive, but there are no outward signs of infection. This stage may last for many years.
Some people will then have no more symptoms, but some 30-50% of untreated patients will progress to tertiary syphilis ("late syphilis"). This involves a slow destruction or breakdown of organic functioning in the central nervous system and blood vessels. The result can be widespread damage to the heart, brain, eyes, nervous system, bones, and joints, as well as mental illness, blindness, severe damage to the heart and aorta, and death.
The tertiary stage is characterized by appearance of infectious granulomas – cell clusters in various tissues. A skin granuloma is referred to as gumma. Such formations disintegrate causing irreversible lesions. For example disintegration of a gumma located in the soft or hard palate results in perforation of the tissues.
Laboratory diagnosis includes a special examination of material from infectious lesions, called a darkfield examination. Blood tests are more commonly used, but syphilis cannot be detected this way until 4 to 6 weeks after the appearance of a chancre.
Congenital syphilis occurs when the fetus is infected in the uterus, and occurs in some 80-85% of cases of early untreated maternal syphilis.
During the primary (hard chancre) stage syphilis in men may be complicated by balanitis, balanoposthitis, inflammatory phimosis, paraphimosis, gangrenization and phagedenas.
The secondary stage may bring such manifestations as syphilitic balding (during the 3d – 5th month of the disease), lesions of bones, joints and periosteum.
During the tertiary (late) syphilis irreversible destructive lesions occur (lesions of hard and soft palate, palatine arches, tongue, pharynx with formation of punctures, bone and periosteal inflammations, osteomielitis, hydroarthrosis and osteoarthrosis, lesions of nervous system, heart and other organs).
Flat white sores can appear in the mouth and/or throat during the second stage of syphilis infection.
Dementia paralytica, or paresis, is a form of syphilis which generally affects patients in their 40s or 50s. Increased behavioral deterioration occurs and the person may be believed to have a "psychiatric illness" or Alzheimer's disease. Symptoms can include convulsions, irritability, difficulty in concentrating, deterioration of memory, defective judgment, headaches, insomnia, fatigue, lethargy, deteriorated hygiene emotional instability, depression, and delusions of grandeur with lack of insight. The patient gradually progresses toward dementia and paralysis.
The gold standard for treatment of syphilis is consecutive daily intramuscular injections with procaine penicillin. The dosage and duration of treatment are determined by the clinical presentation, e.g. chancre, secondary mucocutaneous manifestations, neurosyphilis, etc. If no clinical staging is possible (as is usually the case), serological staging determines the treatment regime. There are a number of treatment options for patients who are allergic to penicillin, who are needle-phobic or who may not comply with a course of daily injections. These treatment options have not been as well studied as the procaine penicillin regimes but are totally acceptable alternatives.
Benzathine penicillin as a single injection will adequately treat primary and secondary syphilis, or sero-positive syphilis with a VDRL or RPR titre of 1:16 or higher. This regime is much more user-friendly than a 10-day use of intramuscular procaine penicillin and compliance is not an issue. This same dose can be given to sexual contacts of the above groups as epidemiological treatment. Because benzathine penicillin does not cross the blood-brain barrier it is only suitable for treating latent syphilis (VDRL or RPR titre < 8) if the C.S.F. is normal. Benzathine penicillin does cross the placenta and is therefore suitable for treating pregnant women with early syphilis.
Non-pregnant patients allergic to penicillin may be treated with doxycycline 300mg daily for 21 days. This regime is considered adequate for treatment of early and late disease, whether symptomatic or latent. This regime would also be appropriate for needle-phobic patients, as would oral amoxycillin 3gm twice daily with 1gm of probenecid for 2 weeks.
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