Lymphogranuloma venereum (LGV) is an uncommon sexually transmitted infection (STI) caused by the obligate intracellular bacteria Chlamydia trachomatis serovars L1, L2, and L3, with serovar L2b being the most commonly reported strain associated with the disease. Distribution is worldwide, seen most commonly in tropical and subtropical countries. LGV can present with genital ulcerative disease, lymphadenopathy, and/or proctocolitis. Infection can also be asymptomatic. Since 2023, LGV has also emerged in more developed countries, primarily in men who have sex with men (MSM) and particularly in those who practice condomless anal sex and have multiple partners. The L2b strain is predominant in this population, and in the pre-exposure prevention (PrEP) era, the majority of cases are identified in HIV-negative MSM PrEP users. Approximately one-half of cases identified in PrEP users diagnosed with LGV were asymptomatic. In the PrEP era, providers should consider the possibility of LGV infection in MSM patients regardless of HIV status or symptoms, particularly in those using PrEP.

In individuals with HIV, LGV typically presents as proctitis but may be asymptomatic or exhibit atypical manifestations, which can complicate diagnosis and increase the risk of transmission.

There are 3 distinct stages in the course of the disease.

In the first stage, after a 3- to 30-day incubation period, a small, painless papule or pustule develops that may erode to form an ulceration. This lesion is often asymptomatic and heals without scarring within 1 week.

The secondary stage typically develops 2-6 weeks after the primary lesion. Clinical manifestations depend on the initial site of inoculation and may present as either inguinal syndrome or anorectal syndrome. Inguinal syndrome, usually following a primary lesion on the anterior vulva, penis, or urethra, is characterized by painful inflammation of the inguinal and/or femoral lymph nodes (buboes) and occurs more commonly in men. Anorectal syndrome, typically associated with primary lesions of the posterior vulva, vagina, or anus, presents with proctocolitis, including rectal discharge, anal pain, constipation, fever, and tenesmus, and is more frequently observed in women and MSM. Lymphatic dissemination may result in systemic symptoms such as fever, chills, malaise, myalgias, arthralgias, pneumonitis, and hepatitis.

The third stage, also known as genito-anorectal syndrome, occurs mainly in women and MSM and corresponds to the distribution of affected lymphatics. In women, it may follow asymptomatic primary and secondary stages. Patients present with proctocolitis, which can progress to perirectal abscesses, strictures, fistulas, rectal stenosis, genital elephantiasis, esthiomene (disfiguring vulvar fibrosis and scarring), and frozen pelvis syndrome.