eMedicine Specialties > Dermatology > Bacterial Infections
Endemic Syphilis
Updated: Feb 13, 2009
Introduction
Background
Features of endemic syphilis have been noted in ancient Africa since history was first recorded. Originally, endemic syphilis was thought to be spread throughout a large geographic area. Through the passage of time, this disease has affixed itself to regions of dry, arid climates.
Endemic syphilis is also known as sibbens (Scotland), radseyege (Scandinavia), siti (Gambia), therlijevo (Croatia), njovera (Southern Rhodesia), frenjak (Balkans), and nonvenereal endemic syphilis (Bejel).
Also see the related eMedicine articles Treponematosis (Endemic Syphilis), Neurosyphilis, and Ocular Manifestations of Syphilis.
Pathophysiology
Different species of the spirochete Treponema cause diverse infections in humans. Treponema pallidum causes venereal syphilis. Treponema carateum and Treponema pertenue cause pinta and yaws, respectively. Endemic syphilis is caused by a spirochete closely related to T pallidum, which is T pallidum subsp endemicum.
Endemic syphilis is transmitted through direct or indirect skin-to-skin or mouth-to-mouth contact of the infected lesion. It occurs predominantly in children aged 2-15 years. Because children are the active transmitters of the disease, infection of all members of a household is very common. The common housefly, Musca domestica, has not been established as a potential vector.
Endemic syphilis has similar pathology and histology as venereal syphilis. However, the effects on the organ systems are different.
The disease has 2 stages, an early stage and a secondary stage. The early stage consists of primary and secondary lesions very similar to those of venereal syphilis. The secondary stage consists of late latent disease and tertiary lesions. Each stage affects different tissues and organs. The primary lesions usually manifest in the oropharynx. Secondary stage lesions can appear as mucous patches on the lips, the palate, and the larynx. Angular stomatitis, condylomata, oral ulcers, and generalized adenopathy can also be seen in the secondary stage. Tertiary and late-stage disease usually develops 6 months to years after inoculation and may manifest as gummas of the skin, the bones, or the cartilage. Neurologic involvement and cardiac involvement are rare.
Frequency
United States
Rare cases of endemic syphilis have been reported in the United States. When reported, the cases are typically seen in immigrants and people coming from endemic areas. Owing to its mode of transmission, endemic syphilis is easily transmitted to new areas. Hygiene; living conditions; and environmental factors, such as the weather, make the disease fastidiously endemic in the United States.
International
Endemic syphilis is extremely common in areas of dry, hot climates. It is also widely spread in rural areas of poor hygiene and education.1
Parts of Africa (eg, Sahel countries [Sudan, Southern Rhodesia, South Africa]), parts of the Middle East (eg, Nomadic/Bedouin tribes of Saudi Arabia, Iraq, and Syria), and parts of Asia (eg, Turkey, Southeast Asia, the Western Pacific) are affected. In these areas, seropositivity in children reaches as high as 40%, and early lesions reportedly affect 2-20% of children.
Mortality/Morbidity
Because the disease rarely manifests clinically significant cardiovascular and neurologic symptoms, mortality is uncommon unless the disease state is highly exaggerated, through either a large inoculum or a devastating immune reaction.Race
Endemic syphilis can affect anyone. Because it is endemic in certain areas of the world, the disease mostly affects ethnicities of those geographic regions.
Sex
Both sexes are equally affected, especially in the pediatric population. This varies with the geographic region. However, in adults, women are slightly more susceptible, probably because they are the primary interactants with children, either as a caregiver or during breastfeeding.
Age
Children aged 2-15 years are most commonly affected; 25% of cases occur before age 6 years, and 55% of cases occur before age 16 years. The remaining 20% of cases occur in adults who are in close contact with children who are infected.2
Clinical
History
Unlike venereal syphilis, endemic syphilis rarely involves the nervous and cardiovascular systems. The clinical manifestation of neurosyphilis is minor and not significant. Congenital syphilis is rarely encountered because the disease can be treated during pregnancy.
Physical
- Primary stage
- The incubation period is 10-90 days.
- Skin lesions resemble the chancres of venereal syphilis. A small, eroded or ulcerated papule is usually asymptomatic.
- Observing a lesion on the nipple of a mother with a suckling child who is infected is not uncommon.
- Primary lesions heal in 1-6 weeks and often go undiagnosed.
- Generalized lymphadenopathy is uncommon since the inoculum is small.
- Secondary stage
- This stage usually consists of macerated, eroded patches on the lips, the tongue, and the tonsils. Hypertrophic condyloma lata can appear in the anogenital area.
- Nontender, generalized lymphadenopathy is common.
- Painful osteoperiostitis in the long bones (eg, tibia) can occur.
- This stage can persist for 6-9 months.
- Angular stomatitis resembling that caused by vitamin B deficiency can be seen.
- Tertiary and late stages
- Destruction of the bone and the cartilage in the formation of gummatous lesions (commonly in the nose) may occur. The gummas can ulcerate and develop chronic serpiginous tracts. Healing results in depigmented scars with a hyperpigmented border.
- Saddle nose deformity and palate perforation can occur.
- Rare atypical involvement of the cardiovascular and nervous systems can occur.
Causes
- T pallidum subsp endemicum, which is transmitted nonvenereally, is the pathogenic organism causing the disease.
- Endemic syphilis is a disease common in areas of poor economic status, education, and personal hygiene. Transmission occurs when skin or mucous membranes come in contact with infected skin lesions. Wearing gloves at all times is imperative for the physician who is examining the lesion.
Differential Diagnoses
Syphilis
Other Problems to Be Considered
Because of the overlap between treponematoses, endemic syphilis has to be correctly differentiated from venereal syphilis, yaws, and pinta. The differential diagnoses of the diseases are very similar, and some features, such as nasopharyngeal lesions, can be difficult to differentiate.
During the early stages, features in endemic syphilis resemble a number of dermatoses, mainly eczema, mycoses, psoriasis, leprosy, herpes simplex, perlèche, and condylomata acuminata.
In the late stages, the features overlap that of malignant tumors, including carcinoma, mycosis fungoides, lupus vulgaris, and lupus erythematosus.3
The mutilating nasopharyngeal lesions can be mistaken for tertiary venereal syphilis, tuberculosis, leprosy, rhinoscleroma, and mucocutaneous leishmaniasis.
Workup
Laboratory Studies
- The best and most reliable method to diagnose endemic syphilis is a thorough clinical history and a complete physical examination. Geographic data, including travel information, is invaluable in establishing the diagnosis.
- Laboratory tests confirm the clinical based diagnosis. These laboratory tests are the same as those used for the diagnosis of venereal syphilis.
- Serologic, morphologic, and biochemical tests are not useful in distinguishing between the types of treponemal infections.
- Serologic tests
- Results of treponemal tests, such as the fluorescence treponemal antibody absorption (FTA-ABS) test, are positive in all stages of the disease.
- Nontreponemal tests, such as the Venereal Disease Research Laboratory (VDRL) test and the rapid plasma reagent (RPR) test, are reactive 2-3 weeks after the onset of the primary lesion. These tests have a sensitivity of 80% in patients with a 2- to 3-month history of symptomatic primary syphilis. In the secondary stage, these tests have a sensitivity of near 100%.
- Dark-field microscopy
- This is the best method to confirm a treponemal disease. Although it does not specify the correct species because of similarities in morphology, the test promptly confirms that the infection is caused by a treponeme.
- Serum is obtained by squeezing the base of the lesion.
Treatment
Medical Care
- Endemic syphilis responds well to penicillin and other treponemicidal drugs. Patients become noninfectious within 24 hours.
- After successful treatment, the titer of nontreponemal tests shows a gradual decline and eventually becomes negative.
- No vaccines for the disease are available.
Medication
The goals of pharmacotherapy are to eradicate the infection, to reduce morbidity, and to prevent complications.
Antibiotics
These agents have the capability to achieve a 100% cure rate. Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.
Penicillin G benzathine (Bicillin LA)
Effectively used to treat primary and secondary endemic syphilis. Tertiary endemic syphilis also responds to treatment but requires a longer time to achieve full effect.
Dosing
Adult
1.2 million units IM once
Pediatric
<10 years: 50,000 U/kg IM once; not to exceed 1.2 million units/dose
>10 years: Administer as in adults
Interactions
Probenecid can increase effects; coadministration of tetracyclines can decrease effects; may increase methotrexate toxicity; may decrease contraceptive efficacy; may interfere with immunological response to live typhoid vaccine; concurrent administration with aminoglycoside therapy may result in inactivation of aminoglycoside (amikacin appears to possess greatest stability in presence of penicillins; in treatment of severely ill patients requiring both penicillin and aminoglycoside therapy, amikacin is aminoglycoside of choice)
Contraindications
Documented hypersensitivity; patients with epilepsy (neurotoxicity is a common feature); patients predisposed to hemorrhage or those receiving anticoagulants
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in impaired renal function, preexisting seizure disorder, or patients with electrolyte abnormalities (contains 1.7 mEq of potassium per million U); can cause acute renal nephritis
Tetracycline (Sumycin)
Can be used in patients allergic to penicillin. Treats gram-positive and gram-negative organisms as well as mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunits.
Dosing
Adult
500 mg PO qid for 15 d
Pediatric
<8 years: Not recommended
>8 years:
Bejel and Yaws: 25-50 mg/kg/d PO divided qid for 15 d
Interactions
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; may enhance agents with neuromuscular blocking effect; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants
Coadministration with retinoids can cause increased intracranial pressure (coadministration contraindicated); administer tetracycline at least 1 h before or 4-6 h after colestipol or cholestyramine; if tetracycline administered concurrently with digoxin, monitor digoxin levels (dosage adjustment for digoxin may be required; risk of interaction may be reduced if given with Lanoxicaps)
Contraindications
Documented hypersensitivity; severe hepatic dysfunction
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Pseudotumor cerebri has been associated with tetracyclines, therefore, possibility for permanent sequelae exists; photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last half of pregnancy through age 8 y) can cause permanent discoloration of teeth; may cause falsely positive urine glucose measurements
Erythromycin (E.E.S., E-Mycin, Ery-Tab)
Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest. For treatment of staphylococcal and streptococcal infections.
In children, age, weight, and severity of infection determine proper dosage. When bid dosing is desired, half-total daily dose may be taken q12h. For more severe infections, double the dose. Can be used in child <8 y.
Dosing
Adult
Erythromycin estolate: 500 mg qid for 10 d
Erythromycin ethylsuccinate: 800 mg 6 times/d or 1600 mg tid for 10 d
Erythromycin stearate: 500 mg 6 times/d for 10 d or 1000 mg tid for 10 d
Pediatric
8 mg/kg PO qid for 15 d
Interactions
May significantly alter metabolism of nonsedating antihistamines and cause serious adverse cardiovascular events; concurrent use of lovastatin and erythromycin may cause rhabdomyolysis in patients who are seriously ill; may increase serum theophylline levels and toxicity; concomitant administration of digoxin may result in elevated serum digoxin levels; coadministration can increase effects of anticoagulants; concurrent use with ergotamine or dihydroergotamine has been associated with acute ergot toxicity; may decrease clearance of triazolam and midazolam; in patients taking other drugs metabolized by cytochrome P-450 system, may be associated with elevations in serum concentrations of those drugs; has demonstrated QTc prolongation in combination with other drugs that prolong the QT interval
Contraindications
Documented hypersensitivity; hepatic impairment; concomitant therapy with astemizole, cisapride, pimozide, or terfenadine
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in liver disease; estolate formulation may cause cholestatic jaundice; GI adverse effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur; neurological symptoms can be potentiated in myasthenia gravis; elderly patients may experience increased susceptibility to torsades de pointes arrhythmias
Follow-up
Prognosis
- Mortality is uncommon (see Mortality/Morbidity).
Patient Education
- Early detection and treatment campaigns aimed at endemic syphilis by health officials are extremely important. Improvement of social and medical conditions and continuing health education help halt the spread and facilitate in the eradication of the disease.
- For excellent patient education resources, visit eMedicine's Sexually Transmitted Diseases Center. Also, see eMedicine's patient education article Syphilis.
References
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Parish JL. Treponemal infections in the pediatric population. Clin Dermatol. Nov-Dec 2000;18(6):687-700. [Medline].
Sharma VK, Kumar B. Malignant syphilis or syphilis simulating malignancy. Int J Dermatol. Sep 1991;30(9):676. [Medline].
Antal GM, Lukehart SA, Meheus AZ. The endemic treponematoses. Microbes Infect. Jan 2002;4(1):83-94. [Medline].
Blount JH, Holmes KK. Epidemiology of syphilis and the non-venereal treponematoses. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York, NY: Academic; 1976:157-76.
Cutler JC. Endemic syphilis, yaws, and pinta. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York, NY: Academic; 1976:365-73.
Engelkens HJ, Niemel PL, van der Sluis JJ, Meheus A, Stolz E. Endemic treponematoses. Part II. Pinta and endemic syphilis. Int J Dermatol. Apr 1991;30(4):231-8. [Medline].
Falabella R. Nonvenereal treponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol. Dec 1994;31(6):1075. [Medline].
Farnsworth N, Rosen T. Endemic treponematosis: review and update. Clin Dermatol. May-Jun 2006;24(3):181-90. [Medline].
Kanan MW, Kandil E. Bejel or non-venereal endemic syphilis. Br J Dermatol. May 1971;84(5):461-4. [Medline].
Knox JM, Musher D, Guzick ND. The pathogenesis of syphilis and the related treponematoses. In: Johnson RC, ed. The Biology of Parasitic Spirochetes. New York, NY: Academic; 1976:249-59.
Koff AB, Rosen T. Nonvenereal treponematoses: yaws, endemic syphilis, and pinta. J Am Acad Dermatol. Oct 1993;29(4):519-35; quiz 536-8. [Medline].
Meheus A, Antal GM. The endemic treponematoses: not yet eradicated. World Health Stat Q. 1992;45(2-3):228-37. [Medline].
Morand JJ, Simon F, Garnotel E, Mahe A, Clity E, Morlain B. [Overview of endemic treponematoses]. Med Trop (Mars). Feb 2006;66(1):15-20. [Medline].
Nsanze H, Lestringant GG, Ameen AM, Lambert JM, Galadari I, Usmani MA. Serologic tests for treponematoses in the United Arab Emirates. Int J Dermatol. Nov 1996;35(11):800-1. [Medline].
Keywords
nonvenereal syphilis of children, sibbens, radseyege, siti, therlijevo, njovera, frenjak, Treponema pallidum subsp endemicum, T pallidum subsp endemicum, nonvenereal endemic syphilis Bejel, non-venereal endemic syphilis Bejel
Contributor Information and Disclosures
Author
Hassan I Galadari, MD, MBBS, Assistant Professor of Dermatology, Faculty of Medicine and Health Sciences, United Arab Emirates University
Hassan I Galadari, MD, MBBS is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, and American Society for Dermatologic Surgery
Disclosure: Nothing to disclose.
Coauthor(s)
Ibrahim Galadari, MD, MB, BCh, MSc, Professor of Dermatology, Department of Internal Medicine, Faculty of Medicine and Health Sciences, United Arab Emirates University
Ibrahim Galadari, MD, MB, BCh, MSc is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Medical Editor
Craig A Elmets, MD, Director of Dermatology, Departments of Dermatology, Pathology, and Environmental Health Sciences; Professor, The Kirklin Clinic, University of Alabama at Birmingham
Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, and Society for Investigative Dermatology
Disclosure: Palomar Medical Technologies Stock None; Merck Consulting fee Independent contractor; Tronox Consulting fee Independent contractor; Amgen Consulting fee Review panel membership; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment
Pharmacy Editor
Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.
Managing Editor
Paul Krusinski, MD, Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont
Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.
CME Editor
Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.
Chief Editor
Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
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