History
The clinical course of LGV consists of the following stages.
First stage (primary LGV)
This stage occurs 3-30 days after inoculation.
Primary LGV begins as a small, painless papule or pustule that may erode to form a small, asymptomatic herpetiform ulcer that usually heals rapidly without scarring.
The most common sites of infection for men include the coronal sulcus, prepuce, glans, and scrotum. Rarely, symptoms of urethritis occur.
The most common sites of infection in women include the posterior vaginal wall, posterior cervix, fourchette, and vulva.
The initial lesion, especially in women, often goes unnoticed by the patient.
Second stage (secondary LGV)
Secondary LGV begins 2-6 weeks after the primary lesion.
This second stage consists of painful regional lymphadenopathy (usually in the inguinal and/or femoral lymph nodes).
Painful, swollen lymph nodes coalesce to form buboes, which may rupture in as many as one third of patients. Those that do not rupture harden, then slowly resolve.
Inguinal lymphadenopathy occurs in only 20-30% of females with LGV; they more typically have involvement of the deep iliac or perirectal nodes and may only present with nonspecific back and/or abdominal pain.
This stage is when most men present and are diagnosed; most women are not diagnosed in this stage because of their lack of inguinal lymphadenopathy.
Constitutional symptoms associated with the second stage include fever, chills, myalgias, and malaise.
Systemic spread may lead to the following conditions:
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Arthritis
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Ocular inflammatory disease
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Cardiac involvement
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Pulmonary involvement
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Aseptic meningitis
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Hepatitis or perihepatitis
Third stage (tertiary LGV)
Tertiary LGV is termed genitoanorectal syndrome.
This condition is more common in women, secondary to their lack of symptoms during the first two stages.
Rectal involvement is more common in men who have sex with men (MSM) and in women who practice anal-receptive intercourse.
Tertiary LGV is characterized by proctocolitis. Although infectious proctitis is more commonly associated with inflammatory bowel disease, sexually transmitted diseases (STDs) must be considered in the work-up of this diagnosis, especially in MSM. In this patient population, the incidence of infectious proctitis attributed to STDs is on the rise.
Fever of unknown origin (FUO)
Symptoms include the following conditions:
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Bloody purulent discharge
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Rectal pain
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Tenesmus
Physical
Large fluctuant buboes or any otherwise unexplained perianal deformity in a young female should suggest a diagnosis of LGV.
First stage (primary LGV)
The initial lesion is usually a small, painless papule, shallow ulcer, or herpetiform lesion in the genital area, which may go unnoticed in the urethra, vagina, or rectum.
Initial lesions may be differentiated from the more common herpetic lesions by the lack of pain associated with the lesion. Differentiation from a syphilitic chancre is more problematic and requires serologic testing.
Second stage (secondary LGV)
Secondary LGV is characterized by painful lymph nodes (usually unilateral) known as buboes.
Enlargement of the inguinal nodes above and the femoral nodes below the inguinal ligament leads to the classic groove sign, which is observed in one third of affected men.
Inguinal lymphadenopathy results from a primary lesion of the anterior vulva, penis, or urethra.
Perirectal or pelvic lymphadenopathy results from a primary lesion involving the posterior vulva, vagina, or anus.
Affected nodes often coalesce and form abscesses, which can rupture and form sinus tracts.
Third stage (tertiary LGV)
Tertiary LGV most often manifests in women.
Patients initially develop proctocolitis.
Patients may present with perirectal fistulas, ulcers, abscesses, strictures, and rectal stenosis.
Hyperplasia of intestinal and perirectal lymphatics may form lymphorrhoids, which are similar to hemorrhoids.
Patients may develop strictures and fistulous tracts secondary to repeated tissue scarring and repair.
Enlargement, thickening, and fibrosis of the labia may occur in women, a condition termed esthiomene.
Chronic lymphatic obstruction may lead to elephantiasis of the genitals.
Penile and scrotal edema and distortion have been termed saxophone penis.
Causes
The L1, L2, and L3 serovars of C trachomatis cause LGV. Risk factors include residing in or visiting endemic areas, practicing anal-receptive intercourse, eschewing condoms, and working in the commercial sex trade.
Complications
Bubo rupture may lead to fistulas and sinus tracts. This complication typically occurs during the first stage (primary LGV) of infection.
Proctocolitis may lead to fissures, fistulas, abscess, scarring, and strictures.
