Venereal lymphogranuloma: causes, symptoms, diagnosis, treatment

Lymphogranuloma venereum (HSV) (synonyms: fourth venereal disease, Nicolas-Favre disease) is a sexually transmitted infection caused by pathogenic microorganisms Chlamydia trachomatis types LI, L2, L3. Lymphogranuloma venereum is not widespread in the world, although it accounts for 2-10% of cases of diseases accompanied by genital ulcers in India and Africa. It is most common in people aged 20-30 years. Men more often seek medical attention with an acute form of lymphogranuloma venereum, while women - with complications at a late stage of the disease.

The most common clinical manifestation of lymphogranulomatosis venereum in heterosexual men is painful inguinal and/or femoral lymphadenopathy, usually unilateral. Women and active homosexual men may present with proctocolitis or inflammatory changes in the perirectal or perianal lymphatic tissue, which may eventually lead to fistulas and strictures. Most untreated patients develop a self-limited genital ulcer, sometimes at the site of inoculation. Diagnosis is usually made by serologic tests and by exclusion of other causes of inguinal lymphadenopathy or genital ulcers.

Neither the degree of infectivity of the pathogen nor the reservoir of the disease are precisely known, although it is believed that transmission of lymphogranuloma venereum occurs mainly through asymptomatic female carriers.

Symptoms of lymphogranuloma venereum. The incubation period is from 3 to 12 days before the onset of the first stage and 10-30 days before the onset of the second stage.

There are 3 stages in the course of lymphogranuloma venereum. After inoculation, a small painless papule or pustule appears, which can erode, forming a small herpetiform ulcer (the first stage of lymphogranuloma venereum). The primary lesion in men is often localized on the neck of the glans penis, frenulum of the foreskin, foreskin, glans and body of the penis, scrotum, in women - on the back wall of the vagina, frenulum of the labia, posterior lip of the cervix and vulva. This lesion usually heals within a week and is often not noticed by patients. At this stage, mucopurulent discharge from the urethra in men and from the cervix in women can be observed.

The second stage of lymphogranuloma venereum occurs within 2-6 weeks after the appearance of the primary lesion and is manifested by painful inflammation of the inguinal and/or femoral lymph nodes.

Lymphogranuloma venereum is primarily a disease of the lymphatic system that progresses to lymphadenitis. Infected macrophages invade regional lymph nodes. This produces a typical picture of unilateral lymph node enlargement (in 65% of patients), infection, and abscess. The painful lymph nodes are called buboes and may coalesce and rupture in a third of patients. In the remaining cases, they become hard, non-purulent masses. Although most buboes heal without complications, some may progress to form chronic fistulas. About a third of patients have a "groove sign" due to enlargement of the inguinal and femoral lymph nodes, located above and below the inguinal ligament, respectively.

Inguinal lymphadenopathy occurs in 20% of women with lymphogranuloma venereum. In women, primary lesions are more common in the rectum, vagina, cervix, or posterior urethra, with involvement of the deep iliac or perirectal lymph nodes.

Lower abdominal or back pain may result. Many women do not develop the characteristic inguinal lymphadenopathy; approximately one-third of them present with signs and symptoms of stage II, while most men present at this stage of the disease. Systemic symptoms such as low-grade fever, chills, malaise, myalgias, and arthralgias are common at this stage of the disease. In addition, systemic dissemination of C. trachomatis occasionally results in arthritis, pneumonia, and perihepatitis. Rare systemic complications include cardiac involvement, aseptic meningitis, and inflammatory eye disease.

The third stage of lymphogranuloma venereum is often called "genitoanorectal syndrome" and is more common in women. Proctitis develops first, followed by a pararectal abscess, strictures, fistulas, and stenosis of the rectum, leading to the formation of "lymphorroidal nodes" (similar to hemorrhoids). If left untreated, chronic lymphangitis leads to the formation of multiple scars, strictures, and fistulas, which can ultimately lead to elephantiasis.

Laboratory testing of smears prepared from purulent discharge and stained with Romanovsky-Giemsa revealed Chlamidia Trachomatis; antibodies to Chlamidia Trachomatis were also detected using ELISA. The results of serological reactions for syphilis were negative.

Laboratory diagnostics. Bacterioscopic method: detection of the pathogen in smears prepared from purulent discharge and stained according to Romanovsky-Giemsa.

Culture method. The diagnosis of LGV can be made by isolating a culture of the microorganism and typing the cells in the sample. The material is best taken from the affected lymph node or affected tissue with a swab. The technique is relatively insensitive: 50% positive even if cyclohexamide-treated McCoy cells or DEEA-treated HeLa cells are used.

Complement fixation reaction in paired sera. The diagnostic value is a reagin titer of 1:64 or a 4-fold increase in the antibody titer after 2 weeks (the so-called "paired sera").

Alternative methods include immunofluorescence assay using monoclonal antibodies and PCR.

The course is unpredictable. Spontaneous remissions often occur.

Treatment of venereous lymphogranuloma. Etiotropic treatment is carried out. It has a good effect in the early stages of the disease. The recommended regimen is doxycycline 100 mg 2 times a day for 21 days. As an alternative regimen, erythromycin is prescribed 500 mg 4 times a day for 21 days.

Treatment is directed at the cause of the disease and at preventing tissue damage, as scarring may occur. If buboes are present, aspiration or incision through intact skin with drainage may be required. Doxycycline is the preferred treatment.

Recommended scheme

Doxycycline 100 mg orally 2 times daily for 21 days.

Alternative scheme

Erythromycin 500 mg orally 4 times daily for 21 days.

The activity of azithromycin against C. trachomatis suggests that this drug may be effective when given in multiple doses for 2 to 3 weeks, but clinical data on its use in this disease are limited.

Follow-up observation

Patients should be observed until symptoms and signs resolve.

Management of sexual partners

Sexual partners of patients with lymphogranulomatosis venereum should be examined, tested for urethral or cervical chlamydial infection, and treated if they had sexual contact with the patient within 30 days before the patient developed symptoms of lymphogranulomatosis venereum.

Special Notes

Pregnancy

In pregnant and lactating women, the erythromycin treatment regimen should be used.

HIV infection

Individuals with HIV infection and Hodgkin's disease venereum should be treated according to the regimens presented earlier. Rare data on the combination of Hodgkin's disease venereum and HIV infection suggest that such patients require longer treatment and that there may be delayed resolution of symptoms.