Lymphogranuloma Venereum


Article Author:
Prashanth Rawla


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Faten Limaiem



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Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
6/26/2019 1:07:05 PM

Introduction

Lymphogranuloma venereum (LGV) is an ulcerative disease of the genital area.[1] Its cause is the gram-negative bacteria Chlamydia trachomatis, especially serovars L1, L2, and L3.[2] It is an uncommon, sexually transmitted disease. It is transmittable by vaginal, oral or anal sex. There are increasing numbers of reports in men who have sex with men.[3][4]

Characteristically, lymphogranuloma venereum has three stages of infection [1]:

  • Primary stage characterized by the development of painless genital ulcer or papules.
  • Secondary stage with the development of unilateral or bilateral tender inguinal and/or femoral lymphadenopathy (also called buboes).
  • Late stage with strictures, fibrosis, and fistulae of the anogenital area.

Etiology

The causative agent is Chlamydia trachomatis, serovars L1, L2, L3.[5] Chlamydia is a ubiquitous ovoid or obligate intracellular bacteria.

Epidemiology

Lymphogranuloma venereum is common in the tropical and subtropical regions around the world. It is rare in the United States.[6] Lymphogranuloma venereum may occur at any age; however, the highest incidence of LGV is in the sexually active population between 15 and 40 years. Lymphogranuloma venereum probably affects both sexes equally although it is more commonly reported in men because early manifestations of LGV are more apparent in men.[7] It is endemic to men who have sex with men. There is a significant association between HIV-infected patients and LGV.[8] Men typically present with the acute form of the disease whereas women often present when they develop complications from later stages of the disease.[7]

Pathophysiology

Lymphogranuloma venereum primarily affects the lymphatic system. Chlamydia trachomatis serovars extend from the primary infection site to the regional lymph nodes and cause a lymphoproliferative reaction, facilitated by binding of Chlamydia trachomatis to the epithelial cells. Binding occurs via heparin sulfate receptors.[9]

Histopathology

Lymph nodes:

Tiny necrotic foci infiltrated by neutrophils represent the earliest microscopic change in an affected node. These enlarge and coalesce to form the stellate abscess that represents the most characteristic feature of this disease. In later stages, epithelioid cells scattered Langhans giant cells, and fibroblasts are seen to line the abscess walls. Confluence of these abscesses is common, and cutaneous sinus tracts may develop. Nodules with dense fibrous walls surrounding amorphous material represent the healing stage.[1]

Rectal biopsies:

Histopathologic examination of rectal biopsies in patients with LGV proctitis usually reveals severe inflammatory changes, and these have been misattributed to inflammatory bowel disease in recent years. Histopathologists should consider LGV in the differential diagnosis when reporting on inflammatory rectal biopsy specimens as a relevant risk behavior might not be provided in the clinical history accompanying the specimen.[1]

History and Physical

Evaluation should involve a thorough history including a sexual history.

Characteristically lymphogranuloma venereum has three stages [10]:

Primary Stage:

  • Begins in 3 to 12 days after exposure or sometimes it may be longer up to 30 days.
  • The patient characteristically develops a painless genital ulcer or papules which are about 1 to 6 mm in size. Sores can also be present in the mouth or throat. An inflammatory reaction can occur at the site of inoculation.
  • This stage often goes unnoticed due to the location of the lesions and as the lesions are usually small and there are no associated symptoms.
  • The lesions resolve or heal spontaneously after few days.

Secondary Stage:

  • The secondary stage presents with the development of unilateral or bilateral tender inguinal and/or femoral lymphadenopathy (also called buboes), which occurs two to six weeks after the primary stage; this is called the inguinal syndrome.
  • An anorectal syndrome also presents which is characterized by proctitis or proctocolitis-like symptoms. Pain during urination, rectal bleeding, pain during passing stools, abdominal pain, anal pain, tenesmus. Generalized symptoms like body aches, headache, and fever can occur during this stage.  This syndrome usually occurs when the transmission is via the anal route.[11]
  • An oral syndrome can occur in people get LGV through the oral route. Cervical lymphadenopathy can occur.
  • There are also reports of systemic complications like pneumonia and hepatitis.[12]

Late Sequelae:

Usually, occur when the disease is left untreated.

  • Necrosis and rupture of the lymph nodes
  • Anogenital fibrosis, and strictures
  • Anal fistulae
  • Elephantiasis of the genital organs can also occur in some cases

Evaluation

Diagnosis is by clinical suspicion. Other causes of genital ulceration and inguinal adenopathy should be excluded.[13]

The basis for a definitive diagnosis of LGV is on serology tests (complement fixation or micro-immunofluorescence) or identification of Chlamydia trachomatis in genital, rectal and lymph node specimens (by culture, nucleic acid amplification test or direct immunofluorescence).[14]

Men who have sex with men who have signs and symptoms of proctocolitis should receive testing for LGV. In these patients testing of rectal specimens with nucleic acid amplification test is the preferred approach.[15][14]

HIV testing should be a consideration in patients with a sexually transmitted disease.

Treatment / Management

The recommended treatment regimen is doxycycline 100 mg orally twice a day given for 21 days.[16]

An alternate regimen is erythromycin 500 mg orally four times a day given for 21 days.

Azithromycin 1 gm orally once weekly for 3 weeks is also an effective alternative regimen.[2]

All patients who are suspected to have LGV (either genito-ulcerative disease with lymphadenopathy or proctocolitis) should be empirically treated for LGV before an official diagnosis is certain.

Pregnant patients can have treatment with erythromycin. Doxycycline and other tetracyclines should be avoided in pregnancy due to the risk of disruption of bone and teeth development.

Patients who have fluctuant or pus-filled buboes can benefit from aspiration of the node, which provides symptomatic relief, although incision and drainage of the nodes are not recommended as it can delay the healing process.[17]

Differential Diagnosis

Sexually transmitted diseases are the first to consider in the differential diagnosis of LGV.

The exclusion of diseases which cause genital ulceration and inguinal adenopathy including herpes simplex, syphilis, chancroid, herpes, and granuloma inguinale will help narrow the diagnosis. 

HIV and lymphoma can also cause generalized lymphadenopathy.

Dermatological conditions and trauma which can cause genital ulcerations should also be in the differential diagnosis.

Prognosis

Prognosis is fair if recognized and treated early.

Complications

Complications usually occur when the disease is left untreated — necrosis and rupture of the lymph nodes, anogenital fibrosis, and strictures, anal fistulae. Elephantiasis of the genital organs can also occur in some cases. Systemic complications like pneumonia and hepatitis also have been reported.[18]

Consultations

In difficult to diagnose and treat cases and infectious disease consultation may be warranted.

Surgical consultation is needed for tertiary or late-stage disease if complications like fistulas or strictures cause damage to the anorectal area.

Deterrence and Patient Education

Patients should receive education regarding the signs and symptoms of early recognition of LGV. Having LGV can increase a patients risk of contracting other sexually transmitted diseases like HIV, hepatitis, syphilis, chancroid, gonorrhea, etc. Patients should learn about the need to use condoms or other protective measures while engaging in sexual activity. Sexual partners of patients who tested positive or probably positive should also be tested, and treatment initiated if necessary. Men who have sex with men should recognize that LGV is prevalent in these populations and should know the signs and symptoms of LGV.

Pearls and Other Issues

Management of Sexual Partners of LGV Patients:

All exposed partners of LGV probable or confirmed patients in the last 60 days should receive testing and empiric treatment with a chlamydial regimen (doxycycline 100mg PO BID for 7 days or 1gm azithromycin one-time dose). Appropriate testing should be done based on the route of transmission and can include either cervical, urethral or rectal specimens. If testing for chlamydia and/or LGV comes back positive, then treatment should be continued to complete a 21-day course. If testing is negative for chlamydia or LGV, then treatment should cease after 7 days.

A diagnosis of LGV should be taken into consideration by gastroenterologists and histologists in patients presenting with proctitis or inguinal lymphadenopathy, particularly in men who have sex with men.[19]

Enhancing Healthcare Team Outcomes

Treatment of LGV affected patients and the recognition of potentially exposed partners requires a coordinated effort from the physicians and nursing staff. Physicians and nurses should educate the patients on the early recognition of signs and symptoms of LGV and other STDs and encourage to seek treatment early to improve outcomes and prevent and complications. Pharmacists should recognize the different medications used for the treatment of LGV and their complications and educate the patients including the risks and benefits of usage of these medications in pregnancy and lactation. A multidisciplinary approach will provide the best long-term results for these patients. [Level V]


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Lymphogranuloma Venereum - Questions

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Which of the following will most likely have Gamna-Favre bodies?



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The groove sign is often seen in individuals with which of the following?



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A 17-year-old sexually active male presents to his primary care provider with complaints of swelling in his right groin area since last one week which is progressively getting more prominent and painful. He also complaints of occasional pain while urinating, occasional lower abdominal pain and some bright red blood in his stool once in a while and a feeling that he has to have a bowel movement frequently. He also mentions that he has been having headaches, body aches, and low-grade fever for one day. He says that he has been sexually active with persons of the same sex since the last three years and does not use any protection while engaging in sexual activity. He does mention that he recently got tested for HIV and hepatitis and both tests were negative. Now he is concerned about his swelling in his right groin area and other associated symptoms. He does mention that he saw a small red, painless lesion near his anal area 3 to 4 weeks back which resolved on its own. Vital signs were blood pressure of 127/76 mmHg, heart rate of 67 bpm, a temperature of 99.0 F, and oxygen saturation of 97% on room air. Physical exam reveals a large tender lymph node on the right side of his groin area. Examination of his anogenital area reveals no ulcers. Which organism is the likely cause of his symptoms?



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If lymphogranuloma venereum (LGV) is suspected because of painful inguinal adenopathy following well-healed, small vulvar ulceration, how can the diagnosis be established?



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What is true about lymphogranuloma venereum (LGV)?



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What is not part of the differential diagnosis for lymphogranuloma venereum?



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A 17-year-old female patient develops a painless papule on her vulva that resolves spontaneously. She does not seek any medical attention as she did not have any other symptoms. Three weeks later she develops painful inguinal lymphadenitis. What is the most likely diagnosis?



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A 29-year-old female presents to the emergency department with complaints of pain and swelling in her left groin area. She mentions that 3 weeks ago she developed a painless red lesion on her vaginal area which resolved spontaneously. The patient also mentions that she is pregnant and currently in her second trimester. On physical exam, tender left-sided inguinal adenopathy is found. The physician confirms the suspicion of possible sexually transmitted infection. Testing confirms the diagnosis of lymphogranuloma venereum. Which of the following is the best treatment for this patient?



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A 25-year-old female presents with complaints of swelling which she has noticed on her right side of her groin since the last week which is slowly increasing in size. She says that whenever she touches the swelling on her groin area, it hurts. She also complains of occasional body aches and low-grade fever for the last two days. She also noticed a small painless red lesion near her vulvar area 3 weeks back which resolved spontaneously. She is sexually active and has 2 active partners. She denies any other past medical history. She has never been tested for HIV. She is scared that she might have an STD. Her last menstrual period was one month back. What is the next best step in the management of this patient?



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A male presents after he noticed pain while defecating. He has had these symptoms from the last week. He says that he saw a small red ulcer which was not painful after he had sex with a new male partner four weeks back. The ulcer developed five days after the encounter and resolved spontaneously. Then he noticed the presenting symptoms starting last week. He is sexually active with men and does not use condoms. He denies any past medical history of sexually transmitted infections. On physical exam, he has tender inguinal lymphadenopathy. Histologic examination shows large, intracytoplasmic basophilic inclusion bodies seen in endothelial cells. Which of the following is the most likely diagnosis?



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A 33-year-old male with a history of sexual activity with men and practicing receptive anal intercourse complains of 10 weeks of pain in his anorectal area and discharge. He also complains of pain during urination, occasional bright red rectal bleeding and pain during passing stools. On examination, there is tender inguinal adenopathy noted. On proctoscopy, there is mucosal inflammation with painless ulcers noted in the perianal area. The patient mentions that he does not have any allergies to any medications. Which of the following is the best initial therapy while awaiting confirmation of the diagnosis?



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Lymphogranuloma Venereum - References

References

Workowski KA,Bolan GA, Sexually transmitted diseases treatment guidelines, 2015. MMWR. Recommendations and reports : Morbidity and mortality weekly report. Recommendations and reports. 2015 Jun 5;     [PubMed]
Mabey D,Peeling RW, Lymphogranuloma venereum. Sexually transmitted infections. 2002 Apr;     [PubMed]
Blank S,Schillinger JA,Harbatkin D, Lymphogranuloma venereum in the industrialised world. Lancet (London, England). 2005 May 7-13;     [PubMed]
Nieuwenhuis RF,Ossewaarde JM,Götz HM,Dees J,Thio HB,Thomeer MG,den Hollander JC,Neumann MH,van der Meijden WI, Resurgence of lymphogranuloma venereum in Western Europe: an outbreak of Chlamydia trachomatis serovar l2 proctitis in The Netherlands among men who have sex with men. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 2004 Oct 1;     [PubMed]
Rönn MM,Ward H, The association between lymphogranuloma venereum and HIV among men who have sex with men: systematic review and meta-analysis. BMC infectious diseases. 2011 Mar 18;     [PubMed]
Chen JC,Stephens RS, Trachoma and LGV biovars of Chlamydia trachomatis share the same glycosaminoglycan-dependent mechanism for infection of eukaryotic cells. Molecular microbiology. 1994 Feb;     [PubMed]
Schachter J, Chlamydial infections. The Western journal of medicine. 1990 Nov;     [PubMed]
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Bachmann LH,Johnson RE,Cheng H,Markowitz L,Papp JR,Palella FJ Jr,Hook EW 3rd, Nucleic acid amplification tests for diagnosis of Neisseria gonorrhoeae and Chlamydia trachomatis rectal infections. Journal of clinical microbiology. 2010 May;     [PubMed]
Meyer T, Diagnostic Procedures to Detect Chlamydia trachomatis Infections. Microorganisms. 2016 Aug 5;     [PubMed]
Handsfield HH, Lymphogranuloma Venereum Treatment and Terminology. Sexually transmitted diseases. 2018 Jun;     [PubMed]
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. International journal of STD     [PubMed]
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Ceovic R,Gulin SJ, Lymphogranuloma venereum: diagnostic and treatment challenges. Infection and drug resistance. 2015     [PubMed]
Su H,Raymond L,Rockey DD,Fischer E,Hackstadt T,Caldwell HD, A recombinant Chlamydia trachomatis major outer membrane protein binds to heparan sulfate receptors on epithelial cells. Proceedings of the National Academy of Sciences of the United States of America. 1996 Oct 1     [PubMed]
Dal Conte I,Mistrangelo M,Cariti C,Chiriotto M,Lucchini A,Vigna M,Morino M,Di Perri G, Lymphogranuloma venereum: an old, forgotten re-emerging systemic disease. Panminerva medica. 2014 Mar     [PubMed]
Harrison T,Som M,Stroup J, Lymphogranuloma venereum proctitis. Proceedings (Baylor University. Medical Center). 2016 Oct     [PubMed]
Stoner BP,Cohen SE, Lymphogranuloma Venereum 2015: Clinical Presentation, Diagnosis, and Treatment. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 2015 Dec 15     [PubMed]
Martin IM,Alexander SA,Ison CA,Macdonald N,McCarthy K,Ward H, Diagnosis of lymphogranuloma venereum from biopsy samples. Gut. 2006 Oct     [PubMed]

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