Etiology
Infection with Chlamydia trachomatis occurs through contact with mucous membranes or abrasions in the skin. Sexual transmission is the most common route, but extragenital sites may be affected when inoculated by nonsexual contact, accidental laboratory inhalation, or when transmitted by exposure to fomites.
Pathophysiology
Chlamydiae are obligate intracellular bacteria. The infective form of Chlamydia trachomatis is the elementary body, which may enter cells by phagocytosis or endocytosis. The organism binds to epithelial cells via the major outer-membrane protein, the OmcB protein, and heparin sulfate receptors.[17]
While other chlamydial infections tend to be confined to the site of inoculation, LGV genotype-specific C trachomatis extends directly to the draining regional lymph nodes, where they proliferate as reticulate bodies within lymphocytes and monocytes. Reticular bodies revert to elementary bodies and the inclusion bodies containing them rupture within the host cell. Consequently, the cell lyses, releases its infective contents and the lifecycle perpetuates with cell-to-cell spread.[18] Epithelial cells, when infected, produce inflammatory cytokines and an initial neutrophilic response. In vitro studies demonstrate that interferon-gamma inhibits replication, but does not terminate the lifecycle.[19]
Inguinal and femoral nodes are the most frequently observed nodes initially involved in heterosexual men. In women and men who have sex with men, lower abdominal pain and back pain are common presenting symptoms associated with involvement of deep pelvic and lumbar lymph nodes. In women, these nodes become involved due to lymphatic spread from the cervix and posterior vaginal wall. Within the lymph nodes, inflammation occurs locally and an inflammatory mass forms when surrounding tissue becomes involved. As the lymphatic inflammation becomes suppurative, abscesses may coalesce or become necrotic. The buboes (inflamed, tender, swollen lymph nodes) that form can rupture spontaneously and create fistulae or sinus tracts. Lymphatic architecture is remodeled by scarring and fibrosis. Subsequent obstruction may lead to genital elephantiasis. Systemic manifestations may occur by direct extension through sinus tracts or bacteremic spread.[20]
Classification
Clinical classification[1]
Three stages of LGV have been identified: primary, secondary, and tertiary.
Primary stage:
Characterized by painless penile or vulvar inflammation and ulceration at the site of inoculation (may be genitals or anus), which spontaneously heals within a few days; this is often not noticed by the patient.
Secondary stage:
The classic inguinal presentation has become a less common finding, but occurs weeks after development of the primary lesion and presents as painful, unilateral, inguinal or femoral lymphadenopathy, possibly with buboes (often referred to as "inguinal syndrome").
When acquired through direct exposure to the rectal mucosa, proctocolitis is another presentation with key symptoms including anorectal pain, rectal bleeding or mucopurulent discharge, diarrhea or constipation, abdominal cramping, reduced anorectal aperture, or tenesmus.
Patients may present with fever, malaise, back or abdominal pain, or arthralgias. Less commonly a "groove sign of Greenblatt" (describes the characteristic sausage-shaped swellings of the inguinal lymph node above the inguinal ligament and the femoral lymph node below the inguinal ligament, where the inguinal ligament forms a groove in between the swellings) may be seen.
Tertiary stage:[2]
Characterized by chronic and progressive edema, which results in enlargement, scarring, and ultimately destructive ulceration of the genitalia.
This is the most common presentation in women due to a lack of symptoms in primary and secondary stages in women.
Sequelae of chronic infection may result in fibrosis, and formation of sinus tracts and strictures of the anogenital tract as abscesses rupture. In women, this may progress to esthiomene (fibrotic genital elephantiasis), or fistulae involving the urethra, vagina, uterus, or rectum. In men, a physical finding known as saxophone penis or penoscrotal elephantiasis has also been described.
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