2.5.3 Lymphogranuloma venereum (LGV)

Grading & Level of Importance: C

Review:
2021

W. Burgdorf, Munich; J.McGrath, London
Revised by A. Salava, E. Hiltunen-Back, A. Ranki, Helsinki

ICD-11

1A80

Synonyms

Chlamydial lymphogranuloma; Frei disease; Durand-Nicolas-Favre disease; climatic bubo.

Epidemiology

In Europe the current LGV epidemic has been detected among MSM, causing mainly proctitis. Some cases of heterosexual LGV have been seen in Europe, most of them were imported from endemic countries (Africa, India). 

Definition

Uro- and ano-genital infection by C. trachomatis, serovars L1-3

Aetiology & Pathogenesis

LGV is caused by the invasive L1-3 strains (serovars) of C. trachomatis that disseminate via underlying connective tissue and spread to regional lymph nodes. Most cases in Europe have been detected among men who have sex with men (MSM).

Signs & Symptoms

Three stages of the infection:

 

1. stage develops after an incubation period of one to four weeks. In the current LGV epidemic among MSM, proctitis is the primary manifestation of infection. Patients complain severe ano-rectal pain, purulent discharge and bleeding from the rectum, tenesmus and constipation. In classical LGV the primary lesion is often an unnoticed, small, painless papule or pustule that erodes to an ulcer and heals within one week.

 

2. stage (inguinal) begins 2 to 6 weeks after the onset of primary lesion. There is painful inflammation of the inguinal and/or femoral lymph nodes causing unilateral enlargement, inflammation, and abscesses (buboes) that may rupture. Constitutional symptoms like low-grade fever, chills, malaise, myalgias and arthralgias may occur.

 

3. stage (ano-genitorectal syndrome) and is more often present in women and MSM. Patients develop proctocolitis followed by perirectal abscesses, fistulas, strictures and stenosis of the rectum.

Classification

See above

Laboratory & other workups

C. trachomatis NAAT test is used for screening and if positive, the diagnosis is confirmed by the detection of LGV biovar-specific C. trachomatis DNA from the same specimen. Many LGV positive men are also HIV-positive, so screening for other STIs including HIV, hepatitis B and hepatitis C should be offered.

Complications

Systemic spread of C. trachomatis occasionally results in arthritis, or (peri) hepatitis. Rare systemic complications include cardiac involvement, aseptic meningitis and ocular inflammatory disease. Ano-genitorectal syndrome with chronic progressive lymphangitis can lead to chronic oedema and sclerosing fibrosis, resulting in strictures and fistulas of the involved region, which can ultimately lead to elephantiasis.

Diagnosis

Based on clinical features and confirmed by microbiological tests

Differential diagnosis

LGV proctitis mimics chronic inflammatory bowel diseases like Crohn’s disease both clinically and histopathologically. Inguinal lymph node enlargements and ulceration can mimic boils, hidradenitis suppurativa and lymphomas.

Prevention & Therapy

First line treatment is doxycycline 100mg twice a day orally for 21 days and second line erythromycin 500mg four times a day orally for 21 days. Fluctuant buboes should be aspirated promptly through healthy adjacent skin. A test of cure for LGV is not considered necessary if the recommended 21-day course of doxycycline is completed.

Special

Sexual partners within the last 3 months are strongly recommended testing for Chlamydia/LGV and epidemiological treatment with antibiotic therapy commenced until Chlamydia/LGV has been excluded in the partner. All LGV cases should be reported according to the national legislation of communicable diseases.

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