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==Causes==
==Causes==
''Chlamydia trachomatis'' is a [[Gram-negative]], [[obligate]] intracellular [[pathogen]] causing [[Lymphogranuloma venereum]].
''Chlamydia trachomatis'' is a [[Gram-negative]], [[obligate]] intracellular [[pathogen]] causing [[Lymphogranuloma venereum]].
==Classification==
Lymphogranuloma venereum (LGV) may be classified into three stages: primary LGV characterized by a self-limited [[papule]], secondary LGV characterized by painful [[lymphadenopathy]], and tertiary LGV (genitoanorectal syndrome) characterized by [[proctocolitis]].
==Differential Diagnosis==
Lymphogranuloma venereum (LGV) must be differentiated from other diseases that cause genital ulcers, [[lymphadenopathy]], or [[proctocolitis]] including [[syphilis]], [[Herpes simplex]], [[Behçet's disease]], [[chancroid]], [[donovanosis]], [[fixed drug eruption]], [[psoriasis]], chlamydial diseases caused by ''C. trachomatis'' [[serovars]] D-K, and diseases characterized with [[colitis]]. [[Sexually transmitted diseases]] characterized as [[genital ulcer diseases]] will present with the most similar symptoms to LGV.


==Epidemiology and Demographics==
==Epidemiology and Demographics==
In the United States, Europe, Australia and most of Asia and South America LGV is generally considered to be a rare [[disease]]. However, a recent outbreak in the Netherlands among gay men has led to an increase of LGV in Europe and the United States. A majority of these patients are HIV co-infected. Since the 2004 Dutch outbreak 341 cases have been reported in the UK and 80 cases in the US, but infectious-disease experts fear the actual number is substantially larger because this form of chlamydia is difficult to diagnose and many physicians are not aware of its existence.
Lymphogranuloma venereum (LGV) is rare in developed countries but the disease has become prevalent in the men who have sex with men (MSM) population. [[Outbreaks]] in MSM have occurred in the United Kingdom, Netherlands, Germany and United States. LGV is seen most commonly in white individuals whose ages range from 15 to 40 years. Males typically present and earlier stages than females.


Soon after the initial Dutch report national and international health authorities launched warning initiatives and multiple LGV cases where identified in several more European countries (Belgium, France, the UK, Germany, Sweden, Italy and Switzerland) and the US and Canada. All cases reported in Amsterdam and France and a considerable part of LGV infections in the UK and Germany are caused by a newly discovered Chlamydia variant L2b, a.k.a the Amsterdam variant. The L2b variant could be traced back and was isolated from anal swabs of men who have sex with men who visited the STI city clinic of San Francisco in 1981. This finding suggests that the recent LGV outbreak among MSM in industrialised countries is a slowly evolving epidemic. As of end 2005, new LGV cases are continued to be reported in the Netherlands and other European countries at rates approaching one or two cases per week in each country.
==Risk Factors==
Risk factors for developing lymphogranuloma venereum include [[Human Immunodeficiency Virus (HIV)|HIV]]-positive serostatus, unprotected sexual intercourse (anal intercourse higher risk than vaginal intercourse) and multiple sex partners.


==Risk Factors==
==Natural History, Complications, and Prognosis==
LGV is more common in men than women. The main risk factor is having multiple sexual partners.
After an incubation period of 3 - 30 days for ''Chlamydia trachomatis'', a [[papule]] develops at the point of [[inoculation]] and may [[Ulcer|ulcerate]]. The lesion is self-limited and heals in approximately a week. [[Lymphadenopathy]] of the [[inguinal]] and [[femoral]] lymph nodes develops 2 - 6 weeks after onset the primary lesion. [[Inguinal]] lymph nodes may develop into fluctuant, suppurative [[buboes]] or nonsuppurative [[abscesses]]. [[Iliac]] and [[perirectal]] lymphadenopathy may also develop in patients with rectal exposure, accompanied by hemorrhagic [[proctocolitis]]. Chronic inflammation may lead to perirectal [[fistulas]] and/or [[strictures]], as well as sclerosing [[fibrosis]] that results in [[elephantiasis]] of genitalia, esthiomene in women, and frozen pelvis syndrome. Systemic spread may result in [[arthritis]], [[hepatitis]] or [[perihepatitis]], [[pneumonitis]], cardiac involvment (rare), [[aseptic meningitis]] (rare), ocular inflammatory disease (rare). Prognosis is poor without treatment. However, spontaneous [[remission]] is possible. Death can occur from bowel obstruction or [[perforation]].


==Diagnosis==
==Diagnosis==
===History and Symptoms===
The most common symptom of primary LGV is a painless [[papule]] or [[ulcer]]. Secondary LGV is characterized by painful [[inguinal]] and/or [[femoral]] [[lymphadenopathy]], or [[Iliac]] and/or [[perirectal]] lymphadenopathy in patients with rectal exposure. [[Proctolcolits]] may also develop at this stage. Tertiary LGV symptoms include Lymphatic and rectal [[fibrosis]], genital [[elephantiasis]], esthiomene (chronic ulcerative disease of vulva), [[edema]], [[tenesmus]] and rectal discharge. LGV may spread systemically and cause the following symptoms: [[fever]], chills, [[malaise]], [[myalgia]], [[arthralgia]], [[arthritis]], [[hepatitis]] or [[perihepatitis]], and [[pneumonitis]].
===Physical Examination===
Primary LGV is characterized by a small, nontender [[papule]] or [[ulcer]]. The primary lesion is typically unnoticed so few patients present at this stage. Majority of patients that do present are male. Secondary LGV is characterized by tender, swollen [[lymph nodes]], typically unilateral, known as [[buboes]]. Enlarged [[inguinal]] and/or [[femoral]] lymph nodes occur after primary lesion of anterior genital area. Enlarged [[iliac]] and/or [[perirectal]] lymph nodes occure after primary lesion of posterior genital area. 20% of patients present with "groove sign". [[Buboes]] may be indurated or draining sinuses. Tertiary LGV is characterized by [perirectal]] [[fistulas]] and/or [[strictures]], ulcerative [[proctitis]], and/or [[Elephantiasis]] of gentials. Males may present with "saxophone [[penis]]". Females may present with with ulceration and thickening of the [[vulva]].
===Laboratory Findings===
===Laboratory Findings===
Because of limitations in a commercially available test, diagnosis is primarily based on clinical findings. Direct identification of the bacteria from a lesion or site of the infection may be possible through testing for [[chlamydia]] but, this would not indicate if the chlamydia infection is LGV. However, the usual chlamydia tests that are available have not been FDA approved for testing rectal specimens. In a patient with rectal signs or symptoms suspicious for LGV, a health care provider can collect a specimen and send the sample to his/her state health department for referral to CDC, which is working with state and local health departments to test specimens and validate diagnostic methods for LGV.
Because of limitations in commercially available tests, clinical presentation, in conjunction to laboratory findings, plays an important role in diagnosis. ''C. trachomatis'' can be identified from lesion swabs, [[bubo]] [[aspirate]], or [[rectal]] [[mucosa]] swabs (in patients with [[proctitis]]) using culture methods, [[Serology|serologic]] testing, and nucleic acid amplification tests (NAATs). Culture is impractical since it is time consuming and lacks sensitivity. Compliment fixation and [[immunofluorescence]] serologic tests are sensitive but only genus specific. NAATs are the most reliable method for detecting ''C. trachomatis'' and real-time [[Polymerase Chain Reaction|PCR]] can detect the LGV-specific [[serovar]].


==Treatment==
==Treatment==
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===Surgery===
===Surgery===
Patients with [[rectal strictures]] as a complication of LGV may need surgery to prevent [[bowel obstruction]] and [[bowel infarction]].
Needle [[aspiration]] or incision and drainage of [[inguinal]] [[buboes]] may be required to prevent [[ulcer]]  or [[sinus]] formation. Patients with [[rectal strictures]] as a complication of LGV may need surgery to prevent [[bowel obstruction]] and [[bowel infarction]].


===Primary Prevention===
===Primary Prevention===
The surest way to avoid transmission of sexually transmitted diseases is to abstain from sexual contact, or to be in a long-term mutually monogamous relationship with a partner who has been tested and is [[asymptomatic]] and uninfected.
Since there is no [[vaccine]] for LGV, methods of primary prevention include: abstaining from sexual activity, limiting number of sexual partners and using a male or female condom. The goal of secondary prevention is to stop the spread of disease. Therefore infected individuals should abstain from sexual intercourse until symptoms reside.
 
Male latex condoms, when used consistently and correctly, may reduce the risk of LGV transmission.  Genital [[ulcer]] diseases can occur in male or female genital areas that may or may not be covered (protected by the condom).
 
Having had LGV and completing treatment does not prevent re-infection. Effective treatment is available and it is important that persons suspected of having LGV be treated as if they have it. Persons who are treated for LGV treatment should abstain from sexual contact until the infection is cleared.


==References==
==References==
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Latest revision as of 18:00, 18 September 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nate Michalak, B.A.

Overview

Lymphogranuloma venereum (LGV), also known as lymphopathia venerea, tropical bubo, climatic bubo, strumous bubo, poradenitis inguinales, Durand-Nicolas-Favre disease, lymphogranuloma inguinale and neekerisankkeri in Finland, is a sexually transmitted disease caused by the invasive serovars L1, L2, or L3 of Chlamydia trachomatis.

Historical Perspective

LGV was first described by Wallace in 1833 and again by Durand, Nicolas, and Favre in 1913.

Pathophysiology

Lymphogranuloma venereum (LGV) is caused by serovars L1, L2, or L3 of the bacterium Chlamydia trachomatis. C. trachomatis is an obligate intracellular pathogen transmitted through vaginal, anal, or oral sexual contact. C. trachomatis alternates between two forms: the infectious elementary body (EB) and noninfectious, replicating reticulate body (RB). EBs enter through skin abrasions or microabrasion, or by crossing mucous membranes. EBs produce major out membrane protein (MOMP), which binds to heparan sulfate receptors on epithelial cells and are then phagocytized forming chlamydial inclusions. EBs begin to differentiate into RBs that start replicating. Endocytosis and bacterial replication cause host cell destruction leading to the formation of a papule at the site of inoculation. EBs and RBs then travel through lymphatics to regional lymph nodes, primarily to inguinal lymph nodes. C. trachomatis continues to replicate within monocytes causing lymphadenopathy and eventually the formation of buboes. Associated conditions include coinfection with other sexually transmitted diseases.

Causes

Chlamydia trachomatis is a Gram-negative, obligate intracellular pathogen causing Lymphogranuloma venereum.

Classification

Lymphogranuloma venereum (LGV) may be classified into three stages: primary LGV characterized by a self-limited papule, secondary LGV characterized by painful lymphadenopathy, and tertiary LGV (genitoanorectal syndrome) characterized by proctocolitis.

Differential Diagnosis

Lymphogranuloma venereum (LGV) must be differentiated from other diseases that cause genital ulcers, lymphadenopathy, or proctocolitis including syphilis, Herpes simplex, Behçet's disease, chancroid, donovanosis, fixed drug eruption, psoriasis, chlamydial diseases caused by C. trachomatis serovars D-K, and diseases characterized with colitis. Sexually transmitted diseases characterized as genital ulcer diseases will present with the most similar symptoms to LGV.

Epidemiology and Demographics

Lymphogranuloma venereum (LGV) is rare in developed countries but the disease has become prevalent in the men who have sex with men (MSM) population. Outbreaks in MSM have occurred in the United Kingdom, Netherlands, Germany and United States. LGV is seen most commonly in white individuals whose ages range from 15 to 40 years. Males typically present and earlier stages than females.

Risk Factors

Risk factors for developing lymphogranuloma venereum include HIV-positive serostatus, unprotected sexual intercourse (anal intercourse higher risk than vaginal intercourse) and multiple sex partners.

Natural History, Complications, and Prognosis

After an incubation period of 3 - 30 days for Chlamydia trachomatis, a papule develops at the point of inoculation and may ulcerate. The lesion is self-limited and heals in approximately a week. Lymphadenopathy of the inguinal and femoral lymph nodes develops 2 - 6 weeks after onset the primary lesion. Inguinal lymph nodes may develop into fluctuant, suppurative buboes or nonsuppurative abscesses. Iliac and perirectal lymphadenopathy may also develop in patients with rectal exposure, accompanied by hemorrhagic proctocolitis. Chronic inflammation may lead to perirectal fistulas and/or strictures, as well as sclerosing fibrosis that results in elephantiasis of genitalia, esthiomene in women, and frozen pelvis syndrome. Systemic spread may result in arthritis, hepatitis or perihepatitis, pneumonitis, cardiac involvment (rare), aseptic meningitis (rare), ocular inflammatory disease (rare). Prognosis is poor without treatment. However, spontaneous remission is possible. Death can occur from bowel obstruction or perforation.

Diagnosis

History and Symptoms

The most common symptom of primary LGV is a painless papule or ulcer. Secondary LGV is characterized by painful inguinal and/or femoral lymphadenopathy, or Iliac and/or perirectal lymphadenopathy in patients with rectal exposure. Proctolcolits may also develop at this stage. Tertiary LGV symptoms include Lymphatic and rectal fibrosis, genital elephantiasis, esthiomene (chronic ulcerative disease of vulva), edema, tenesmus and rectal discharge. LGV may spread systemically and cause the following symptoms: fever, chills, malaise, myalgia, arthralgia, arthritis, hepatitis or perihepatitis, and pneumonitis.

Physical Examination

Primary LGV is characterized by a small, nontender papule or ulcer. The primary lesion is typically unnoticed so few patients present at this stage. Majority of patients that do present are male. Secondary LGV is characterized by tender, swollen lymph nodes, typically unilateral, known as buboes. Enlarged inguinal and/or femoral lymph nodes occur after primary lesion of anterior genital area. Enlarged iliac and/or perirectal lymph nodes occure after primary lesion of posterior genital area. 20% of patients present with "groove sign". Buboes may be indurated or draining sinuses. Tertiary LGV is characterized by [perirectal]] fistulas and/or strictures, ulcerative proctitis, and/or Elephantiasis of gentials. Males may present with "saxophone penis". Females may present with with ulceration and thickening of the vulva.

Laboratory Findings

Because of limitations in commercially available tests, clinical presentation, in conjunction to laboratory findings, plays an important role in diagnosis. C. trachomatis can be identified from lesion swabs, bubo aspirate, or rectal mucosa swabs (in patients with proctitis) using culture methods, serologic testing, and nucleic acid amplification tests (NAATs). Culture is impractical since it is time consuming and lacks sensitivity. Compliment fixation and immunofluorescence serologic tests are sensitive but only genus specific. NAATs are the most reliable method for detecting C. trachomatis and real-time PCR can detect the LGV-specific serovar.

Treatment

Medical Therapy

There is no vaccine against the bacteria. LGV can be treated with three weeks of antibiotics. CDC STD Treatment Guidelines recommend the use of doxycyline, twice a day for 21 days. An alternative treatment is erythromycin base or azithromycin. The health care provider will determine which is best.

Surgery

Needle aspiration or incision and drainage of inguinal buboes may be required to prevent ulcer or sinus formation. Patients with rectal strictures as a complication of LGV may need surgery to prevent bowel obstruction and bowel infarction.

Primary Prevention

Since there is no vaccine for LGV, methods of primary prevention include: abstaining from sexual activity, limiting number of sexual partners and using a male or female condom. The goal of secondary prevention is to stop the spread of disease. Therefore infected individuals should abstain from sexual intercourse until symptoms reside.

References

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