3/12/2018 Lymphogranuloma Venereum (LGV): Background, Pathophysiology, Epidemiology

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Lymphogranuloma Venereum (LGV)

Updated: May 15, 2017
Author: Pamela Arsove, MD, FACEP; Chief Editor: Pranatharthi Haran Chandrasekar, MBBS, MD
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OVERVIEW

Background
Lymphogranuloma venereum (LGV) is an uncommon sexually transmitted disease (STD) caused by
Chlamydia trachomatis. LGV is endemic in certain areas of Africa, Southeast Asia, India, the
Caribbean, and South America. It is rare in industrialized countries, but in the last 10 years has been
increasingly recognized in North America, Europe, and the United Kingdom as causing outbreaks of
proctitis among men who have sex with men (MSM). [1, 2, 3, 4]

LGV is a subtype of genital ulcer diseases that include other STDs, such as HSV-2, syphilis, and
chancroid. This condition is characterized by self-limited genital papules or ulcers followed by painful
inguinal and/or femoral lymphadenopathy, which may be the only clinical manifestation at
presentation. Patients with LGV may also present with rectal ulcerations and symptoms of
proctocolitis, especially among patients participating in receptive anal intercourse. In these cases,
rectal pain, discharge, and bleeding may be confused with other GI conditions such as colitis. [5] If left
untreated, disfiguring ulceration and enlargement of the external genitalia, and subsequent lymphatic
obstruction, may occur.

Pathophysiology
C trachomatis is an obligate intracellular bacterium. Of the 15 known clinical serotypes, only the L1,
L2, and L3 serotypes cause LGV. These serotypes are more virulent and invasive compared to other
chlamydial serotypes. Infection occurs after direct contact with the skin or mucous membranes of an
infected partner. The organism does not penetrate intact skin. The organism then travels by
lymphatics to regional lymph nodes, where it replicates within macrophages and causes systemic
disease. While transmission is predominantly sexual, cases of transmission through laboratory
accidents, fomites, and nonsexual contact have been reported.

The L2b serovar has been identified to play a more important role than previously expected. After the
diagnosis of 92 cases of LGV in the Netherlands among MSM, Schachter evaluated samples obtained
from rectal swabs between 1979 and 1985 from patients infected with HIV in San Francisco and
between 2000 and 2005 in Amsterdam. [6] The study revealed the same serotype circulating among
patients with HIV and LGV 20-25 years ago. This indicates the L2b serovar has been present and
unrecognized for many years.

LGV occurs in 3 stages. The first stage, which is often unrecognized, consists of a rapidly healing,
painless genital papule or pustule. The second stage, consisting of painful inguinal lymphadenopathy,
occurs 2-6 weeks after the primary lesion. The third stage, which is more common in women and
MSM, may occur many years after the original infection and is characterized by proctocolitis.

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Epidemiology
Frequency
United States

LGV is historically a rare disease in developed countries. Since 2003, however, sporadic outbreaks of
LGV proctitis have been reported among MSM in North America, Europe, and Australia. [7, 8] However,
in the United States, the true incidence is unknown because national reporting of LGV ended in 1995.

Currently, no universal surveillance data exist for this disease. Twenty four states still mandate
reporting of LGV cases to the Center for Disease Control (CDC), which provides limited data for
disease prevalance. Since 1972, rates of LGV have steadily declined, with 113 known cases reported
to the CDC in 1997. In November 2004, the CDC began offering assistance to test for LGV in the
United States. Between November 2004 and January 2006, LGV was identified in 180 specimens,
with 27 specimens identified as being obtained from homosexual males.

A study published in 2011 reporting LGV surveillance data from multiple sites in the United States
found that less than 1% of the samples obtained from rectal swabs of MSM that were positive for C
trachomatis tested positive for LGV. [9]

International

LGV is an uncommon disease, although it may account for 2-10% of patients with genital ulcer
disease in selected areas of India and Africa. [10] The disease is most commonly found in areas of the
Caribbean, Central America, Southeast Asia, and Africa. Since 2003, however, the emergence of
documented LGV infections, mostly among MSM, but also in women, has prompted increased
surveillance and reporting of this disease in developed countries. [11, 12] Proctitis is reemerging as a
presentation of LGV in developing countries. [13]

After a cluster of 92 cases was identified in the Netherlands between 2003 and 2004 (where fewer
than 5 cases were reported yearly), [14] many countries have begun active surveillance for LGV, and
an increasing number of cases has been identified. Evidence exists that among MSM, LGV may be
endemic in the UK; between 2004-2008, LGV was documented in 854 isolates by the National
Reference Center there. [15, 16, 17, 18, 19, 20, 21]

Mortality/Morbidity

With appropriate treatment, the disease is easily eradicated. Death is a rare complication but could
possibly result from a small bowel obstruction or perforation secondary to rectal scarring.

Morbidity is common, especially during the third stage of the disease, and includes such conditions as
proctocolitis, perirectal fissures, abscesses, strictures, and rectal stenosis. A chronic inflammatory
response may lead to hyperplasia of the intestinal and perirectal lymphatics, causing lymphorrhoids,
which are similar to hemorrhoids. Strictures and fistulous tracts may lead to chronic lymphatic
obstruction, resulting in elephantiasis, thickening or fibrosis of the labia, and edema or gross distortion
of the penis and scrotum. Reports show an association between adenocarcinoma (primarily rectal
adenocarcinoma) and chronic untreated LGV.

Race

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In North America and Europe, most reported cases of LGV have been identified among white males
infected with HIV who acquired the condition after having sex with other men after travel or living in
endemic areas, and typically after having multiple anonymous sexual contacts.

Sex
LGV is an STD and probably affects both sexes equally, although it is more commonly reported in
men. This predilection may be because early manifestations of LGV are more apparent in men and
are thus diagnosed more readily. Men typically present with the acute form of the disease, whereas
women often present later, after developing complications from late disease.

Most cases in Europe and North America have been identified among white, frequently HIV-positive
MSM patients presenting with proctitis. [20, 22, 23, 24]

Age

LGV may affect any age but has a peak incidence in the sexually active population aged 15-40 years.

Clinical Presentation

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Contributor Information and Disclosures

Author

Pamela Arsove, MD, FACEP Associate Residency Director, Department of Emergency Medicine,
Hofstra Northshore Long Island Jewish School of Medicine; Attending Physician, Department of
Emergency Medicine, Long Island Jewish Medical Center; Assistant Professor, Department of
Emergency Medicine, Northshore Long Island Jewish School of Medicine
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3/12/2018 Lymphogranuloma Venereum (LGV): Background, Pathophysiology, Epidemiology

Pamela Arsove, MD, FACEP is a member of the following medical societies: American College of
Emergency Physicians, American Medical Association, Phi Beta Kappa, Society for Academic
Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Barbara Edwards, MD Associate Physician, Division of Infectious Diseases, Department of Medicine,

Long Island Jewish Medical Center; Assistant Professor, Department of Medicine, Albert Einstein
College of Medicine of Yeshiva University

Barbara Edwards, MD is a member of the following medical societies: American College of

Physicians, Infectious Diseases Society of America, Society for Healthcare Epidemiology of America

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical
Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Charles V Sanders, MD Edgar Hull Professor and Chairman, Department of Internal Medicine,
Professor of Microbiology, Immunology and Parasitology, Louisiana State University School of
Medicine at New Orleans; Medical Director, Medicine Hospital Center, Charity Hospital and Medical
Center of Louisiana at New Orleans; Consulting Staff, Ochsner Medical Center

Charles V Sanders, MD is a member of the following medical societies: American College of

Physicians, Alliance for the Prudent Use of Antibiotics, The Foundation for AIDS Research, Southern
Society for Clinical Investigation, Southwestern Association of Clinical Microbiology, Association of
Professors of Medicine, Association for Professionals in Infection Control and Epidemiology, American
Clinical and Climatological Association, Infectious Disease Society for Obstetrics and Gynecology,
Orleans Parish Medical Society, Southeastern Clinical Club, American Association for the
Advancement of Science, Alpha Omega Alpha, American Association of University Professors,
American Association for Physician Leadership, American Federation for Medical Research, American
Geriatrics Society, American Lung Association, American Medical Association, American Society for
Microbiology, American Thoracic Society, American Venereal Disease Association, Association of
American Medical Colleges, Association of American Physicians, Infectious Diseases Society of
America, Louisiana State Medical Society, Royal Society of Medicine, Sigma Xi, Society of General
Internal Medicine, Southern Medical Association

Disclosure: Received royalty from Baxter International for other.

Chief Editor

Pranatharthi Haran Chandrasekar, MBBS, MD Professor, Chief of Infectious Disease, Department

of Internal Medicine, Wayne State University School of Medicine

Pranatharthi Haran Chandrasekar, MBBS, MD is a member of the following medical societies:

American College of Physicians, American Society for Microbiology, International
Immunocompromised Host Society, Infectious Diseases Society of America

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Disclosure: Nothing to disclose.

Acknowledgements

Kenneth C Earhart, MD Deputy Head, Disease Surveillance Program, United States Naval Medical
Research Unit #3

Kenneth C Earhart, MD is a member of the following medical societies: American College of

Physicians, American Society of Tropical Medicine and Hygiene, Infectious Diseases Society of
America, and Undersea and Hyperbaric Medical Society

Disclosure: Nothing to disclose.

Alexandre F Migala, DO Staff Physician, Department of Emergency Medicine, Denton Regional

Medical Center

Disclosure: Nothing to disclose.

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