Lymphogranuloma Venereum

Lymphogranuloma venereum (LGV) is caused by the L1, L2, and L3 serovars of Chlamydia trachomatis, distinct from the D through K serovars causing uncomplicated genitourinary chlamydia, and characterized by a specifically more invasive, lymphotropic disease course.

Historically endemic mainly to parts of Africa, Asia, and South America, but LGV proctitis outbreaks have become increasingly recognized among men who have sex with men in high income settings over the past two decades, and should be actively considered in this population presenting with proctitis.

Disease progresses through three stages, though presentation varies by anatomical site of inoculation.

The primary stage features a small, often unnoticed, painless papule or ulcer at the inoculation site, healing within days.

The secondary stage (the most common presentation prompting clinical attention) follows 2 to 6 weeks later: in the classic inguinal syndrome, painful, unilateral inguinal and/or femoral lymphadenopathy develops, with nodes above and below the inguinal ligament producing the characteristic "groove sign", and buboes may suppurate and form draining sinuses if untreated.

In the increasingly recognized anorectal syndrome (from receptive anal intercourse), a hemorrhagic proctitis develops, with rectal pain, discharge, bleeding, tenesmus, and constipation, which can closely mimic inflammatory bowel disease both clinically and on sigmoidoscopy.

The tertiary stage (if untreated) causes chronic lymphatic obstruction, genital elephantiasis, and rectal strictures or fistulae.

NAAT testing confirms Chlamydia trachomatis, but standard chlamydia NAAT does not distinguish LGV serovars from non-LGV serovars.

Where LGV is clinically suspected (proctitis, particularly in a man who has sex with men, or the classic inguinal syndrome), a positive chlamydia NAAT should prompt specific LGV serovar-specific PCR testing (genotyping) where available. Where such testing is not accessible, give empirical LGV treatment based on clinical suspicion.

Bubo aspiration (never incision and drainage, given sinus tract risk) for culture or PCR where fluctuant nodes are present.

• Doxycycline 100 mg PO bd for 21 days is first line, a notably longer course than for uncomplicated chlamydia given the more invasive disease process
• Alternative: erythromycin base 500 mg PO qds for 21 days, used particularly in pregnancy where doxycycline is contraindicated (azithromycin weekly for 3 weeks, 1 g PO once weekly, is an alternative pregnancy-safe option per some guidelines, though evidence is more limited than for doxycycline in non-pregnant patients)
• Fluctuant buboes should be aspirated through adjacent healthy skin as needed for symptomatic relief, avoiding incision and drainage given the risk of sinus tract formation
• Sexual partners in the preceding 60 days should be tested and treated empirically with a standard chlamydia regimen (doxycycline 100 mg PO bd for 7 days is sufficient for asymptomatic partners without confirmed LGV)
• Co-testing for HIV, syphilis, and gonorrhea given shared risk context, and given LGV proctitis outbreaks have shown high rates of HIV coinfection in affected populations

Referral: sexual health services for partner notification and full STI screening; gastroenterology if rectal stricture develops or the presentation is difficult to distinguish from inflammatory bowel disease.