Lymphogranuloma Venereum (LGV)

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

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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

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.

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