Lymphogranuloma Venereum: Causes, Symptoms, Treatment, Prognosis, Complications, Diagnosis

Lymphogranuloma venereum or LGV is a sexually transmitted bacterial infection that is often characterized by a small, often asymptomatic skin lesion, followed by regional lymphadenopathy in the pelvis or the groin. Without proper treatment, Lymphogranuloma venereum may cause obstruction of lymph flow and chronic swelling of the genital tissues. More about the causes, symptoms, diagnosis and treatments of lymphogranuloma venereum are discussed in the following array of the article.

Lymphogranuloma venereum or LGV is a rare systemic sexually transmitted disease that is caused by three unique strains of Chlamydia trachomatis. In the U.S, about 300 cases are reported every year. Incidence and prevalence is increasing slightly in the US in men having sex with men.

Lymphogranuloma venereum is present as painless vesicular or ulcerative lesions on the external genitalia. These symptoms are seen in early disease followed by tender inguinal or femoral lymphadenopathy, usually unilateral. If left untreated, severe anogenital inflammation and scarring may result. Generally the Gastrointestinal, Lymphatic, Immunogic and Reproductive systems are affected by lymphogranuloma venereum.

Lymphogranuloma venereum is also known by some other names like Tropical bubo, climatic bubo, Strumous bubo, Lymphogranuloma inguinale, Poradenitis inguinalis, Duran-Nicolas-Favre disease, etc.

Lymphogranuloma venereum or LGV is more common is the central and South America than in North America. It is even more common in men than in women.

Lymphogranuloma venereum is a chronic disease caused by Serotypes L1, L2, and L3 of the bacteria Chlamydia trachomatis. These serotypes differ from the chlamydial serotypes that cause trachoma, inclusion urethritis, conjunctivitis and cervicitis, because they can invade and reproduce in the regional lymph nodes. It is reported that there is a higher risk of getting Lymphogranuloma venereum in people having unprotected intercourse, anal intercourse, prostitution, men to men sex, HIV(+ve) and people residing in tropical and developing countries.

Symptoms of Lymphogranuloma venereum or LGV can begin in a few days to a month after coming in contact with the bacteria. The lymphogranuloma venereum symptoms can be studied under the following categories, depending on the site of entry of the infectious organisms or the sex contact site and the stage of disease progression:

Inoculation at the mucous lining of external sex organs like penis and vagina can cause the inguinal syndrome named after the formation of abscesses or buboes in the groin or the inguinal region, where draining lymph nodes are located. These lymphogranuloma venereum signs generally appear from 3 days to one month after the exposure.

The Rectal syndrome in lymphogranuloma venereum arises in case the infection takes place through the rectal mucosa (via anal sex) and is primarily characterized by protocolitis symptoms.

Pharyngeal syndrome begins after infection of pharyngeal tissue, and buboes in the neck can occur. This is pretty rare.

There are mainly three stages of lymphogranuloma venereum progression and symptoms of the disease vary in different stages.

Primary Stage of Lymphogranuloma Venereum: Here, superficial painless lesions like vesicles, papules, ulcers or erosions appear on the external genitalia, in the area of exposure. It can occur in 3 days to one month after the exposure. These lesions disappear in some days without leaving scars.

Secondary Stage of Lymphogranuloma Venereum: In this stage of lymphogranuloma venereum, the inguinal syndrome or hemorrhagic proctitis can occur. Femoral lymph glands can also be involved often unilaterally. This is often predominant in men. Fever, headache, chills and myalgias can be common in the secondary stage of lymphogranuloma venereum. In case of Inguinal syndrome, buboes begin as a mass of firm, tender, matted and enlarged lymph nodes, other unilateral and eventually involving the overlying skin with adhesions and erythema. There is a severe groin pain due to bubo enlargement. The buboes may become fluctuant and rupture in one or two weeks, relieving the pain; but leaving fistulas to drain and form firm inguinal masses. In case of Proctitis, there is anal pruritus and a mucous rectal discharge, rectal pain, several discrete superficial ulcerations with irregular borders, etc.

Tertiary Stage of Lymphogranuloma Venereum: Tertiary stage is the Anogenital stage where there is lymphatic obstruction or scarring, genitalia or anorectal canal inflammation, etc. This is common in women and in men having sex with other men. There may be either perianal growths or lymphoid tissue resembling hemorrhoids, due to lymphatic obstruction. Perirectal abscesses, rectovaginal fistulas, anal fistulas and rectal strictures may also occur in this stage of lymphogranuloma venereum.

Your doctor will examine you and ask about your medical and sexual history. Below are some of the tests that your doctor might prescribe you to diagnose Lymphogranuloma venereum.

Where possible, both swab and sera samples must be submitted for suspected cases of Lyphohranuloma venereum. Serum tests are only useful in case the LGV has become invasive(i.e. in the secondary and tertiary stage). Given below are some of the Initial Laboratory Tests done for diagnosing Lyphohranuloma venereum:

• Genotyping of Lymphogranuloma venereum or LGV by DNA sequencing or RFLP is definitive and it differentiates Lymphogranuloma venereum fro other Chlamydial strains.
• Cultures from a swab of a primary lesion may grow Chlamydia, however; genotyping is required for differentiating LGV from other chlamydial strains.
• NAAT or Nucleic acid amplification tests for Chlamydia are not specific to LGV and are not approved by FDA for rectal samples. Positive NAAT samples can be sent for LGV genotyping.
• Urine can be tested with Nucleic acid applification tests and positives can be sent for LGV genotyping.
• Serum immunoglobulin M microimmunoflurescence or MIF testing is more readily available, but is not definitive of Lymphogranuloma venereum.
• Serum antibody levels to L1, L2, L3 serovars of Chlamydia trachomatis also measured using complement fixation, although cross-reactivity with other chlamydial organisms could be possible.

Direct Fluorescent antibody or DFA test, PCR of likely infected areas and pus, are also used sometimes. DFA test for the L-type Serovar of Chlamydial trachomatis is the most sensitive and specific test for diagnosis of Lyphohranuloma venereum. However, it is not readily available. If PCR or Polymerase chain reaction tests on infected material are positive, subsequent restriction endonuclease pattern analysis of the amplified outer membrane protein A gene can be done so as to determine the genotype.

In recent times, a fast real-time PCR( TaqMan analysis) has been developed to diagnose Lymphogranuloma venereum. With this method of testing, an accurate diagnosis of the condition, is feasible within a day. However, it must be noted that one type of testing may not be thorough enough.

Imaging is generally required to clarify or define complications or to exclude other diagnoses. CT scan for retroperitoneal adenitis may be done.

In most uncomplicated cases, outpatient oral medications are all that are required for LGV. Consider treating empirically for LGV, is specific diagnosis testing is not available for patients with compatible clinical syndrome like genital ulcer disease, proctocolitis etc.

First Line of Medication: Doxycycline 100 mg PO b.i.d for 21 days, in case of the acute condition of Lymphphranuloma venereum. You can consider a longer course of the therapy for chronic or relapsing cases.

Second Line of Medication: Erythromycin base 500 mg q.i.d for 21 days or azithromycin 1 g PO once in a week for 3 weeks, sulfisoxazole 500 mg PO q.i.d for 21 days and chloramphenicol or rifampin have been used to treat Lymphogranuloma Venereum.

Pain medications like NSAIDs or Nonsteroidal anti-inflammatory drugs, as required can be given to the patient suffering from lymphogranuloma venereum.

Note: In order to treat pregnant or nursing women, use erythromycin regimen, as doxycyline is contraindicated in pregnancy.

In case of the acute bubonic stage of lymphogranuloma venereum, nodes should be aspirated via intact skin for diagnostic purposes, and this may improve lymphogranuloma venereum symptoms. Nodes may also be incised and drained for diagnostic purposes, and also to possibly prevent femoral or inguinal ulcerations.

However; there may be delayed healing here.

Patients should be observed until the signs and symptoms of LGV resolve and routine chlamydial tests are negative. Serology should not be used to monitor treatment response, as the duration of antibody response has not yet been defined. The patient should be monitored for fever and bubo pain that usually abate within two days after starting antibiotics. In case of persistent fever, monitor closely for the complications like abscess or super infection. Patient also needs to be monitored for surgical complications. Dual infections with other STDs are common and thus, appropriate monitoring should be performed, especially for gonorrhea, hepatitis B, Hepatitis C, Syphilis and HIV.

Tetracyclines should be taken on an empty stomach except for doxycycline, which can be taken with food. LGV is a sexually transmitted disease and thus, the patient should be counseled about other STDs and safe sex practices. Patient should abstain from sexual contact or intercourse, until the treatment is complete.

Scarring, including possible ureteral or bowel obstruction, gross destruction of the anal canal, anal sphincter, etc. may be some of the complications associated with Lymphogranuloma vereneum. Squamous cell carcinoma has been associated with Lymphogranuloma vereneum.

Prognosis of lymphogranuloma venereum is good when this condition is dealt with early treatment and complete resolution of symptoms is possible, in case treatment is undertaken before scarring. There may be relapse if there is a reinfection and/or inadequate treatment.

You must know that if you have been in contact with someone who may have an STD, including LGV, if you develop symptoms of Lymphophranuloma venereum, etc. you must call your medical provider to be properly diagnosed and treated well. You can prevent LGV by your safer sex behaviours, which may actually reduce the risk of getting this disease. Use of proper condoms, greatly reduces the risk of catching a sexually transmitted infection.