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 but most commonly seen in tropical and subtropical countries. LGV can present with genital ulcerative disease, lymphadenopathy, and/or proctocolitis. There are also reports of asymptomatic infection.

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

Stage 1:
After a 3- to 30-day incubation period, a small, painless papule or pustule develops on the genital area. Most often, it will erode and ulcerate. This lesion is often asymptomatic and heals without scarring within 1 week. Some patients may be unaware of this occurrence.

Stage 2:
The second stage occurs 2-6 weeks after the primary lesion and reflects the spread of the organism from the initial site to regional lymph nodes. When the primary lesion is located on the anterior vulva or urethra, the infection presents as inguinal buboes (painful inflammation of the inguinal lymph nodes), which become fluctuant and rupture. If the lesion is on the posterior vulva, vagina, or anus, it manifests as anorectal syndrome, with proctocolitis characterized by rectal discharge, anal pain, constipation, fever, and tenesmus. Patients may also experience systemic symptoms such as fatigue, headache, and weight loss. This is typically the stage when most patients seek medical attention.

Stage 3:
The third stage, also known as genito-anorectal syndrome, may follow asymptomatic primary and secondary stages in women. 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.

In patients 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.