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Lymphogranuloma venereum is a sexually transmitted condition that is caused by the L1, L2 and L3 serotypes of Chlamydia trachomatis, which is an obligate intracellular bacterial agent. The condition is most commonly observed in tropical and subtropical regions of the world, although recently outbreaks in Europe and North America were observed.
Lymphogranuloma venereum is a chronic and progressive disease. The clinical presentation of the disease may vary according to the patient’s gender, mode of sexual contact, as well as the stage of the disease. Accordingly, the clinical features are usually grouped into three stages. Asymptomatic cases of lymphogranuloma venereum also have to be taken into account.
After an average incubation period of 3-30 days, the primary lesion presents as a transient papule, herpetiform ulcer, or sometimes as nodular ulceration. In men, this lesion is most often located at the coronal sulcus, while in women characteristic localizations are posterior vaginal wall, cervix or vulva.
This primary lesion is self-limiting, which means it may pass unnoticed by the affected individual. It can be distinguished from the more frequent herpetic lesions by the absence of pain; however, adequate differentiation from chancre seen in syphilis necessitates serologic testing.
Mucopurulent discharge from cervix, urethra or rectum is sometimes present (depending on the inoculation site). In men who have sex with men (MSM), a primary manifestation of lymphogranuloma venereum infection is hemorrhagic proctitis, albeit this presentation may also arise in women who had rectal exposure. Certain serovars of causative agent may cause pharyngeal infection.
The second stage of the disease (also known as inguinal syndrome) is observed within 2-6 weeks after primary lesion development, although it may be delayed for as long as half a year. This stage of the disease is characterized by tender and enlarged regional lymph nodes, which is known as “bubo.”
This inguinal bubo is present only on one side of the body in two-thirds of all the cases. As the nodes get larger, the patient experiences grave pain in the groin, often coupled with walking difficulties. When periadenitis sets in approximately two weeks later, the glands become fixed and adherent to the superimposed skin.
In cases of both inguinal and femoral involvement, lymph nodes can be separated by the inguinal ligament (also known as Poupart’s ligament), which gives rise to the phenomenon known as “Groove sign.” This feature is pathognomonic of lymphogranuloma venereum, but is observed in only 20 percent of cases.
Third stage of the disease (also known as genito-anorectal syndrome) is seen in 25 percent of untreated patients, particularly in women who were asymptomatic in previous two stages, but also in MSM who engage in receptive anal sex due to the location of the lymphatics.
In this stage chronic inflammatory response is provoked, that results tissue destruction and, subsequently, the formation of fistulas, perirectal abscesses, strictures, rectal stenosis and scarring. Hemorrhoid-like swelling of obstructed lymphatic tissue may also ensue, which is known as “lymphorrhoids.”
Such sequelae can then cause genital elephantiasis, chronic vulvar ulcerative disease with disfiguring scarring (esthiomene), as well as frozen pelvis syndrome. Furthermore, scrotal and penile edema with distortion that may be seen in this stage of the disease have been dubbed “saxophone penis.” Megacolon is also a possible consequence of lymphogranuloma venereum proctitis.
Although disease manifestations in the third stage are quite dramatic, they are luckily observed rarely (even within current outbreaks within MSM populations). However, appropriate and timely diagnosis is pivotal for early treatment and prevention of potential late complications of the condition.