History
Primary lymphogranuloma venereum (LGV)
LGV has an incubation period of about 1-4 weeks; after which the primary stage may occur.
During the initial stage, a painless papule or pustule that erodes into a herpetiform ulcer may form.
If the primary lesion is in the urethra, mucopurulent discharge may occur.
The most common sites of primary infection in men include the coronal sulcus, frenulum, prepuce, penis, urethra, glans, and scrotum.
In women, the most common sites of the primary lesion include the posterior vaginal wall, fourchette, posterior lip of the cervix, and vulva.
The primary lesion is seen in one third of affected men but rarely is found in affected women.
Primary lesions of the mouth can result from orogenital intercourse.
Secondary LGV
If the primary stage is left undetected, undiagnosed, or untreated the secondary stage of LGV can occur. The incubation period for the secondary stage is typically 2-6 weeks, but it may be up to 6 months. The secondary stage of LGV is characterized by tender, enlarged, unilateral lymph nodes, known as buboes.
Patients may also present with constitutional symptoms, such as fever, headache, malaise, chills, nausea, vomiting, and arthralgias.
Tertiary LGV
In the tertiary stage symptoms are caused from chronic inflammation of the genital, anal, and rectal lymph nodes, depending on where initial inoculation occurred. Inflammation of the rectum progresses to include the colon, leading to the classical proctocolitis of this late stage. Symptoms of protocolitis can include anal pruritus, bloody mucopurulent rectal discharge, fever, rectal pain, tenesmus, constipation, pencil-thin stools, and weight loss. Symptoms mimic chronic inflammatory bowel disease, such as Crohn's disease, which can cause misdiagnosis.
During the tertiary stage, anogenital strictures, anal fistulas, necrosis of the lymph nodes, and elephantiasis of the genital organs can also develop due to the chronic inflammation. [12]
Physical
Usually, LGV is considered based on physical findings of large fluctuant buboes or draining sinuses. The presence of rectal stricture and/or perineal deformity in a young woman is highly suggestive of LGV.
Primary LGV
The initial lesion may be a painless papule, pustule, shallow ulcer, or herpetiform grouping of lesions.
A cordlike lymphangitis of the dorsal penis may develop in primary LGV. This may progress to the formation of a solitary, large, tender lymphoid nodule, or bubonulus. These bubonuli may rupture to form sinuses and/or fistulas.
Secondary LGV
Buboes, which are enlarged, tender, regional lymph nodes, may be present. Buboes are usually unilateral, but occasionally may be bilateral. The location of lymph node involvement is related directly to the site of the primary lesion. Inguinal lymphadenopathy occurs if the primary lesion involves the anterior vulva, penis, or urethra. Perirectal and pelvic lymphadenopathy results if the primary lesion involves the posterior vulva, vagina, or anus. Lymphadenitis of the submandibular and cervical glands occurs if the site of primary inoculation is the mouth.
Seventy-five percent of all patients have deep iliac nodal involvement, which seldom suppurates.
When LGV includes inguinal lymph node involvement, a groove sign may be seen. The groove sign develops when lymphadenopathy of the inguinal nodes above, and femoral nodes below the inguinal ligament coalesce. This sign, however, is only seen in about 20% of patients affected, with the majority being men.
Two thirds of patients with inguinal involvement have unilateral inguinal bubo formation with edema and erythema of the overlying skin. Often these nodes coalesce to form stellate abscesses. One third of these abscesses rupture; two thirds involute. Prior to rupture, the skin overlying the buboes may become a dark, bluish-gray color. After a buboe ruptures, pain decreases; however, a discharge may continue for weeks to months with the formation of a fistula or sinus tract.
Cutaneous manifestations may accompany infection, including erythema multiforme, scarlatiniform eruption, urticaria, and, in 10% of cases, erythema nodosum.
Complications of LGV that may be noted on physical examination include arthritis, conjunctivitis, and hepatomegaly. Pericarditis, pneumonia, and meningoencephalitis rarely occur.
Tertiary LGV
This stage is characterized by proctocolitis from chronic inflammation of the rectum that spreads to the colon.
On examination of the rectum, lymphorrhoids or perianal condylomata may be seen. These structures appear similar to hemorrhoids and are the result of an obstruction of lymphatics. They are composed of dilated lymph vessels with perilymphatic inflammation.
Rectal examination at this stage also may reveal a granular mucosa and palpable, enlarged lymph nodes under the bowel wall. Stricture usually occurs 2-5 cm above the anocutaneous margin, and digital examination above the stricture may reveal smooth healthy mucosa.
Lymph node fibrosis and granulomas are typical in very late stages. In women, esthiomene (eating away) occurs, which results in hypertrophic, chronic granulomatous enlargement of the vulva and subsequent ulceration. This may not appear for 1-20 years after the primary infection. In men, elephantiasis of the genitalia may occur.
Causes
The causal organism is Chlamydia trachomatis, serovars L1, L2, L2b, and L3.
Serovar L2 (including L2b) is most common, however serovar-specific serologic tests of C. trachomatis are not widely available.
Risk factors are similar to risks for transmission of other sexually transmitted infections. Risk factors include the following:
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Unprotected sex
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Anal sex
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Residing in or visiting certain developing countries, where LGV is endemic
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Prostitution
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HIV
Complications
When treated early, complications from LGV are rare. However, if LGV is left untreated the tertiary stage can result. The tertiary stage is characterized by proctocolitis. Complications associated with proctocolitis from LGV may include anal or vaginal fistulas, colonic perforation, anal strictures, and elephantiasis of the genitalia.
