Article 159: Overview and Symptoms of Lymphogranuloma Venereum

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Lymphogranuloma venereum

Lymphogranuloma venereum, also known as inguinal lymphogranuloma, is a chronic sexually transmitted disease caused by Chlamydia trachomatis serotypes L1, L2, and L3. This disease is primarily transmitted through sexual contact, and its clinical manifestations include genital ulcers, swollen and suppurating inguinal lymph nodes, and in later stages, elephantiasis of the external genitalia and rectal stenosis.

What are the symptoms?

1. Early lesions: inguinal lymph nodes and genital, anorectal stages. Incubation period is 1-4 weeks or longer.

(1) Primary lesion: Approximately one-third to one-half of male patients have a primary lesion, which often goes unnoticed. The primary lesion manifests as a single small papule or vesicle on the genitals, which subsequently erodes and may form an ulcer. The lesion has neat edges, is surrounded by redness, and is painless and non-itchy. In men, it commonly occurs on the coronal sulcus, foreskin, penis, glans, and urethra. The primary lesion is generally painless, resolves within 1 to 3 weeks, and leaves no scars.

(2) Lymphadenopathy: Local lymph node enlargement occurs 3-6 weeks after infection and 1-3 weeks after the primary lesion. It mainly affects the inguinal lymph nodes, with about 2/3 of cases being unilateral and the other 1/3 bilateral. The nodules are infiltrative and hard, with red or purplish-red skin overlying them and wrinkled, arranged in a sausage-like pattern along the groin. As the disease progresses, the lymph nodes on both sides of the inguinal ligament are affected and become grooved, a condition known as the groove sign. About 2/3 of the nodules may rupture, forming multiple fistulas, resembling a spray bottle in appearance, with a pale yellow serous or bloody purulent discharge. About 1/4 of the nodules do not rupture and may spontaneously resolve within 8-12 weeks; a few nodules become persistent sclerotic masses. Male patients may also have involvement of the femoral and iliac lymph nodes. Those with deviant sexual behavior may have involvement of lymph nodes in other parts of the body. If there is oral-genital sexual activity with an infected person, swelling of the submandibular lymph nodes may occur, which is quite likely to be considered as lymphoma or Hornerkin's disease.

(3) Proctitis: Homosexuals or those with paraphilia may present with hemorrhagic proctitis (purulent discharge and bleeding). Examination may reveal inflammation, congestion, localized sloughing or granulation tissue of the rectal mucosa, which may lead to perirectal abscess, rectovaginal fistula and rectal stricture.

2. Secondary Stage Lesions: This disease is systemic. While lymph node involvement occurs, systemic symptoms such as fever, night sweats, malaise, weight loss, headache, migratory arthritis, polyarthritis, myalgia, hepatosplenomegaly, pseudomeningitis, and conjunctivitis may also appear. Skin manifestations include erythema multiforme, erythema nodosum, scarlet fever-like rash, papulopustular lesions, and photosensitivity. Male homosexual patients may develop genital-anorectal syndrome. Early stages of this syndrome are caused by direct inoculation or rupture of perirectal lymphadenitis, leading to edema, bleeding, and sloughing of the anal and rectal mucosa, resulting in diarrhea, tenesmus, abdominal pain, and alternating constipation. In later stages, tubular or annular rectal stenosis may occur near the anal ring, as well as rectovaginal and/or anal fistulas and perirectal abscesses. Elephantiasis-like swelling and ulceration may occur in the penis and scrotum. Oral-genital sexual intercourse may result in ulcerative glossitis and lymphadenopathy. Malignant transformation may also occur in later stages.

3. Stage III (late stage) lesions: The main manifestations are general weakness and local disfigurement, mostly caused by rectal stenosis and lymphatic circulation disorders. Men sometimes develop urethral fistulas and genital edema.

What tests should be done?

1. Complement fixation test: This is an important serological diagnostic method for this disease, detecting antibodies against Chlamydia trachomatis and Chlamydia psittaci. Because Chlamydia infection is common in the population, a positive result on this test is helpful for diagnosis, but it cannot be relied upon to confirm the diagnosis. Patients typically have high serum titers, often 1:64 or higher, while serum titers are lower (1:16~1:32) in conjunctivitis caused by Chlamydia trachomatis infection. Generally, a serum titer of 1:8 or 1:6 is suggestive for the diagnosis of this disease, while 1:64 or higher is diagnostically significant. Serum titers decrease during the recovery period. Furthermore, the results of serological tests do not completely parallel the response to antibiotic treatment.

2. Microimmunofluorescence assay: It can detect specific antibodies against different serotypes of Chlamydia and is more sensitive and specific than the complement fixation assay. However, due to limitations in experimental conditions, it is not yet widely used.

3. Pathogen culture: Aspiration of enlarged lymph nodes should be inoculated into the yolk sac of a chicken embryo, or tissue (cell) culture should be performed, or the lymph nodes should be inoculated into mice. Positive results are diagnostically valuable. Bacterial culture and Gram staining of smears are also necessary to rule out lymph node inflammation caused by Staphylococcus or other bacteria.

4. Biopsy: Take tissue samples from skin, mucous membrane lesions or lymph nodes to prepare sections and observe their pathological changes, which can be helpful for diagnosis.

5. Other: Hypergammaglobulinemia, reversed albumin/globulin ratio, elevated IgA and IgG, mild anemia, leukocytosis, accelerated erythrocyte sedimentation rate, false positive syphilis serological test, positive cryoprecipitate and rheumatoid factor, etc.