eMedicine Specialties > Infectious Diseases > Sexually Transmitted Diseases

Lymphogranuloma Venereum (LGV)

Author: Alexandre F Migala, DO, Staff Physician, Department of Emergency Medicine, Denton Regional Medical Center
Contributor Information and Disclosures

Updated: May 15, 2009

Introduction

Background

Lymphogranuloma venereum (LGV) is an uncommon sexually transmitted disease (STD) caused by Chlamydia trachomatis. This condition is characterized by extremely painful inguinal lymphadenopathy.

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 they replicate within macrophages and elicit systemic disease. While transmission is predominantly sexual, cases of transmission through laboratory accidents, fomites, and nonsexual contact have been reported.

The L2b serovar may have been identified to play a more important role than previously expected. After the diagnosis of 92 cases of LGV in the Netherlands among men who have had sex with men, 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.¹ 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, may occur many years after the original infection and is characterized by proctocolitis.

Frequency

United States

Rates of LGV have steadily declined since 1972, with 113 known cases reported in 1997. The true incidence is not known because LGV is not a reported sexually transmitted disease. 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.

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. The disease is most commonly found in areas of the Caribbean, Central America, Southeast Asia, and Africa. Historically rare in Europe, a cluster of 92 cases was identified in the Netherlands between 2003 and 2004, where fewer than 5 cases are usually reported yearly.² In many countries, active surveillance for LGV began in 2004 as a result of the increased number of cases being identified.^3,4,5,6,7

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 have been identified among white HIV-positive men who have sex with men presenting with proctitis.^8,9,10

Age

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

Clinical

History

The clinical course of LGV consists of the following stages:

• First stage (primary LGV)
  • This stage occurs 3-30 days after inoculation.
  • Primary LGV begins as a small, painless papule or pustule that may erode to form a small, asymptomatic herpetiform ulcer that usually heals rapidly without scarring.
  • The most common sites of infection for men include the coronal sulcus, prepuce, glans, and scrotum. Rarely, symptoms of urethritis occur.
  • The most common sites of infection in women include the posterior vaginal wall, posterior cervix, fourchette, and vulva.
  • The initial lesion, especially in women, often goes unnoticed by the patient.
• Second stage (secondary LGV)
  • Secondary LGV begins 2-6 weeks after the primary lesion.
  • This second stage consists of painful regional lymphadenopathy (usually in the inguinal and/or femoral lymph nodes).
  • Painful, swollen lymph nodes coalesce to form buboes, which may rupture in as many as one third of patients. Those that do not rupture harden, then slowly resolve.
  • Inguinal lymphadenopathy occurs in only 20-30% of females with LGV; they more typically have involvement of the deep iliac or perirectal nodes and may only present with nonspecific back and/or abdominal pain.
  • This stage is when most men present and are diagnosed; most women are not diagnosed in this stage because of their lack of inguinal lymphadenopathy.
  • Constitutional symptoms associated with the second stage include fever, chills, myalgias, and malaise.
  • Systemic spread may lead to the following conditions:
    • Arthritis
    • Ocular inflammatory disease
    • Cardiac involvement
    • Pulmonary involvement
    • Aseptic meningitis
    • Hepatitis or perihepatitis
• Third stage (tertiary LGV)
  • Tertiary LGV is termed genitoanorectal syndrome.
  • This condition is more common in women, secondary to their lack of symptoms during the first two stages.
  • Rectal involvement is more common in men who have sex with men and in women who practice anal-receptive intercourse.
  • Tertiary LGV is characterized by proctocolitis.
  • Symptoms include the following conditions:
    • Malaise
    • Weight loss
    • Bloody purulent discharge
    • Fever
    • Rectal pain
    • Tenesmus

Physical

Large fluctuant buboes or any otherwise unexplained perianal deformity in a young female should suggest a diagnosis of LGV.

• First stage (primary LGV)
  • The initial lesion is usually a small, unnoticed painless papule, shallow ulcer, or herpetiform lesion in the genital area.
  • Initial lesions may be differentiated from the more common herpetic lesions by the lack of pain associated with the lesion. Differentiation from a syphilitic chancre is more problematic and requires serologic testing.
• Second stage (secondary LGV)
  • Secondary LGV is characterized by painful lymph nodes (usually unilateral) known as buboes.
  • Enlargement of the inguinal nodes above and the femoral nodes below the inguinal ligament leads to the classic groove sign, which is observed in one third of affected men.
  • Inguinal lymphadenopathy results from a primary lesion of the anterior vulva, penis, or urethra.
  • Perirectal or pelvic lymphadenopathy results from a primary lesion involving the posterior vulva, vagina, or anus.
  • Affected nodes often coalesce and form abscesses, which can rupture and form sinus tracts.
• Third stage (tertiary LGV)
  • Tertiary LGV most often manifests in women.
  • Patients initially develop proctocolitis.
  • Patients may present with perirectal fistulas, abscesses, strictures, and rectal stenosis.
  • Hyperplasia of intestinal and perirectal lymphatics may form lymphorrhoids, which are similar to hemorrhoids.
  • Patients may develop strictures and fistulous tracts secondary to repeated tissue scarring and repair.
  • Enlargement, thickening, and fibrosis of the labia may occur in women, a condition termed esthiomene.
  • Chronic lymphatic obstruction may lead to elephantiasis of the genitals.
  • Penile and scrotal edema and distortion have been termed saxophone penis.

Causes

The L1, L2, and L3 serovars of C trachomatis cause LGV. Risk factors include residing in or visiting endemic areas, practicing anal-receptive intercourse, eschewing condoms, and working in the commercial sex trade.

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