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Synonyms: LGV, Durand-Nicholas-Favre disease, lymphopathia venereum, lymphogranuloma inguinale, tropical bubo, poradenitis inguinales
- This disease is due to infection with the L1, L2 or L3 serovars of Chlamydia trachomatis.
- Unlike genitourinary chlamydial infection which infects squamocolumnar epithelial cells, these serovars cause infection of mononuclear phagocytes in the lymphatic system.
- The disease was largely confined to tropical regions of the world but there are now outbreaks arising locally in Europe (particularly The Netherlands), America and the UK, predominantly affecting men who have sex with men (MSM). This is largely rectal infection presenting with proctitis.
- Cases in the developed world are largely due to the L2b serovar.
- A new LGV variant causing severe proctitis has emerged and has been designated L2c.
- The disease is endemic in East and West Africa, India, the Caribbean, South America and Southeast Asia.
- There are no reliable figures for population prevalence. However, studies of MSM attending four genitourinary medicine (GUM) clinics in the UK for sexual health screening found a prevalence of 0.9% LGV positivity in rectal swabs and 0.4% positivity in urethral swabs.
- Public Health England data showed a significant increase in cases since 2003. The prevalence peaked in 2010 at 543 new cases. In 2012, the figure had reduced somewhat to 395 cases.
- The epidemic in the UK has largely been confined to the metropolitan London area and Brighton but smaller clusters have occurred in other areas - eg, Edinburgh.
- Unprotected sexual intercourse
- Receptive anal intercourse
- Insertive oral intercourse
- Sexual contacts in endemic areas
- Multiple sexual partners
- Male gender
- Anal enema use
The clinical presentation is divided into primary, secondary and tertiary patterns.
Primary lymphogranuloma venereum (LGV)
- Primary LGV presentation is seen in about one third of infected men,but rarely in women.
- It occurs three days to three weeks after exposure.
- It usually presents with a painless papule or shallow ulcer/erosion.
- There may be groups of lesions resembling herpes infection.
- Symptoms of urethritis may occur.
- In men it is usually the coronal sulcus, frenulum, penile shaft, foreskin, glans, scrotum, urethra or anus that are affected.
- Men may develop a penile lymphangitis of the dorsal penile shaft, with cord-like thickening.
- A tender nodule may form in the regional lymph glands which can undergo rupture or sinus formation.
- When women do display symptoms of primary LGV, it affects the posterior vaginal wall, posterior lip of cervix, vulva and fourchette.
- Oral cases may occur in men and women following oral sexual intercourse.
- This usually occurs 10-30 days after exposure but may take several months to develop.
- Buboes (grossly enlarged tender nodes) form in the regional lymph drainage.
- There may be symptoms of systemic illness such as fever, headache, nausea, vomiting, lethargy and arthralgia.
- Buboes affect either the inguinal, pelvic or perirectal lymph nodes, depending on the original site of genital infection and may be unilateral or bilateral.
- If oral infection occurs then the submaxillary and cervical lymph glands are affected.
- The groove sign may occur, particularly in men, due to separation of the enlarged inguinal and femoral lymph nodes by the inguinal ligament. This sign is present in about a fifth of male cases. However, it has also been associated with non-Hodgkin's lymphoma.
- There is usually erythema and induration of skin overlying the enlarged nodes and there may be rupture of the buboes with sinus or fistula formation.
- The skin may be affected by erythema multiforme, urticaria, erythema nodosum or scarlatiniform rash.
- There may (rarely) be signs of conjunctivitis, hepatomegaly, meningoencephalitis, pericarditis, pneumonia and arthritis.
- This late presentation can occur up to 20 years after infection.
- There is usually proctocolitis, which can be confused with other causes of distal colonic inflammation.
- Patients may complain of anal itching, bloody mucopurulent anal discharge, rectal pain and tenesmus, passage of very thin stools with constipation or weight loss.
- Swollen haemorrhoid-like structures, due to lymphatic obstruction, may be seen at the rectal margin.
- Digital rectal examination or proctoscopy may reveal a granular mucosa and enlarged nodes beneath it.
- There may be rectal fibrosis and stricture in advanced cases (reversible with treatment) and elephantiasis of the genitals in men.
- Esthiomene - an 'eating away' of the genitalia - may affect women. There is chronic hypertrophy and granulomatous enlargement of the vulva with ulceration and erosion.
Depends on the stage of disease.
Primary and secondary disease resembles
- Genital herpes.
- Cat scratch disease (Bartonella henselae infection).
- Chancroid (Haemophilus ducreyi - another tropical sexually transmitted infection (STI)).
- Donovanosis, also known as granuloma inguinale (Klebsiella granulomatis - another tropical STI).
- Bubonic plague.
- Mycobacterial infections.
- Malignant causes of lymphadenopathy - eg, Hodgkin's disease.
- Anal/rectal carcinoma.
- Lymphatic filariasis.
- Inflammatory bowel disease.
- Other causes of inguinal lymphadenopathy and genital ulceration must be considered and ruled out.
- Full screening for STIs should be carried out if possible, preferably via a GUM clinic.
- Samples for culture or analysis may be collected from percutaneous drainage of buboes, or from exudate of ulcer base or rectal tissue.
- Complement fixation (CF) testing has largely been replaced by more sensitive and specific nucleic acid amplification tests (NAATs).
- Polymerase chain reaction (PCR) assays, strand displacement amplification (SDA) or transcription mediated amplification (TMA) have the highest specificity and sensitivity and are increasingly being used to reach a definitive diagnosis,.
- CT imaging may be used to assess the extent of lymphadenopathy and to look for alternative causes.
- Sigmoidoscopy/colonoscopy with tissue biopsy may be needed to diagnose the cause of anorectal symptoms. Tissue histology can be nonspecific.
- First-line treatment is usually with doxycycline 100 mg twice-daily for 21 days. Protocols using doxycycline are successful both in those who are and are not co-infected with HIV.
- Evidence for other antibiotics is scarce but azithromycin may be better than erythromycin.
- A case was reported of proctitis and inguinal buboes treated with doxycycline 21 days, azithromycin 20 days and moxifloxacin for a further 12 days because of progressive worsening of inguinal symptoms. Despite extensive antibiotic treatment, the inguinal lymphogranuloma lesions persisted; however, the patient recovered spontaneously after three months.
- Buboes may be drained percutaneously to relieve symptoms.
- Surgical excision is best avoided due to the risk of sinus or fistula formation.
- Patients with rectal stricture or other advanced complications may require surgical intervention.
- Patients should refrain from unprotected sexual intercourse until they and any contacts have completed treatment and follow-up.
- Follow-up should take place within 1-2 weeks for early infection, including MSM proctitis, but may take up to 3-6 weeks for long-standing infections or sequelae.
- If diagnosed in the primary/secondary stages, full cure is expected with appropriate antibiotic therapy. The problem of emerging resistance to doxycycline needs to be considered in patients who are not responding to treatment.
- Tertiary cases may have long-term complications despite bacteriological cure.
- Infection provides no significant immunity to future re-infection and relapse of infection after treatment may occur in some cases.
- Rupture of bubo with sinus or fistula formation
- Fibrosis/deformation of penis
- Cervicitis or salpingitis in women
- Colonic obstruction due to rectal stricture
- Awareness of disease in the developed world.
- Surveillance and testing in GUM clinics/opportunistically in primary care.
- Practice of safe sex.
- Contact tracing of confirmed cases, where possible.
Further reading and references
Heiligenberg M, Verweij SP, Speksnijder AG, et al; No evidence for LGV transmission among heterosexuals in Amsterdam, the Netherlands. BMC Res Notes. 2014 Jun 107(1):355.
Ronn M, Hughes G, White P, et al; Characteristics of LGV repeaters: analysis of LGV surveillance data. Sex Transm Infect. 2014 Jun90(4):275-8. doi: 10.1136/sextrans-2013-051386. Epub 2014 Jan 15.
Martin-Iguacel R, Llibre JM, Nielsen H, et al; Lymphogranuloma venereum proctocolitis: a silent endemic disease in men who have sex with men in industrialised countries. Eur J Clin Microbiol Infect Dis. 2010 Aug29(8):917-25. doi: 10.1007/s10096-010-0959-2. Epub 2010 May 28.
de Vries HJ, Zingoni A, Kreuter A, et al; 2013 European guideline on the management of lymphogranuloma venereum. J Eur Acad Dermatol Venereol. 2014 Mar 24. doi: 10.1111/jdv.12461.
Unemo M et al; Laboratory diagnosis of sexually transmitted infections, including human immunodeficiency virus, WHO, 2013.
Ward H, Alexander S, Carder C, et al; The prevalence of lymphogranuloma venereum infection in men who have sex with men: results of a multicentre case finding study. Sex Transm Infect. 2009 Jun85(3):173-5. doi: 10.1136/sti.2008.035311. Epub 2009 Feb 15.
Latest LGV surveillance data, 2003-2012; Public Health England
Macdonald N, Sullivan AK, French P, et al; Risk factors for rectal lymphogranuloma venereum in gay men: results of a multicentre case-control study in the UK. Sex Transm Infect. 2014 Jun90(4):262-8. doi: 10.1136/sextrans-2013-051404. Epub 2014 Feb 3.
Waalboer R, van der Snoek EM, van der Meijden WI, et al; Analysis of rectal Chlamydia trachomatis serovar distribution including L2 (lymphogranuloma venereum) at the Erasmus MC STI clinic, Rotterdam. Sex Transm Infect. 2006 Jun82(3):207-11.
White J, O'Farrell N, Daniels D; 2013 UK National Guideline for the management of lymphogranuloma venereum: Clinical Effectiveness Group of the British Association for Sexual Health and HIV (CEG/BASHH) Guideline development group. Int J STD AIDS. 2013 Aug24(8):593-601. doi: 10.1177/0956462413482811. Epub 2013 Jul 25.
Nair PS, Nanda KG, Jayapalan S; The "sign of groove", a new cutaneous sign of internal malignancy. Indian J Dermatol Venereol Leprol. 2007 Mar-Apr73(2):141.
Soni S, Srirajaskanthan R, Lucas SB, et al; Lymphogranuloma venereum proctitis masquerading as inflammatory bowel disease in Aliment Pharmacol Ther. 2010 Mar 25.
Vivekanandarajah A, Krishnarasa B, Hurford M, et al; Kikuchi's Disease: A Rare Cause of Fever and Lymphadenopathy. Clin Med Insights Pathol. 20125:7-10. doi: 10.4137/CPath.S8685. Epub 2012 Feb 26.
Martin IM, Alexander SA, Ison CA, et al; Diagnosis of lymphogranuloma venereum from biopsy samples. Gut. 2006 Oct55(10):1522-3.
McLean CA, Stoner BP, Workowski KA; Treatment of lymphogranuloma venereum. Clin Infect Dis. 2007 Apr 144 Suppl 3:S147-52.
Vall-Mayans M, Isaksson J, Caballero E, et al; Bubonic lymphogranuloma venereum with multidrug treatment failure. Int J STD AIDS. 2014 Mar25(4):306-8. doi: 10.1177/0956462413501158. Epub 2013 Aug 28.
Mechai F, de Barbeyrac B, Aoun O, et al; Doxycycline failure in lymphogranuloma venereum. Sex Transm Infect. 2010 Aug86(4):278-9. doi: 10.1136/sti.2009.042093.
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