eMedicine Specialties > Emergency Medicine > Infectious Diseases
Lymphogranuloma Venereum
Updated: Aug 17, 2009
Introduction
Background
Lymphogranuloma venereum (LGV) is a sexually transmitted disease that primarily infects the lymphatics.
The disease originally was described in 1833 by Wallace. It was defined as a clinical and pathological entity in 1913 by Durand, Nicolas, and Favre.
LGV synonyms include lymphopathia venerea, tropical bubo, climatic bubo, strumous bubo, poradenitis inguinales, Durand-Nicolas-Favre disease, and lymphogranuloma inguinale.
Pathophysiology
LGV is caused by serovars L1, L2, and L3 of Chlamydia trachomatis. It gains entrance through skin breaks and abrasions, or it crosses the epithelial cells of mucous membranes. The organism travels via the lymphatics to multiply within mononuclear phagocytes in regional lymph nodes.
Transmission is predominantly sexual. However, transmission by fomites, nonsexual personal contact, and laboratory accidents has been documented. The creation of aerosols of this organism has been associated with infection and pulmonary symptoms.
LGV occurs in 3 stages. The majority of LGV infections in the primary and secondary stages may go undetected.
The primary stage is marked by the formation of a painless herpetiform ulceration at the site of inoculation.
The secondary stage is classically described as the inguinal syndrome in men, characterized by painful inguinal lymphadenitis and associated constitutional symptoms.
- Tender inguinal lymphadenopathy, usually unilateral, is the most common clinical manifestation.
- Lymphatic drainage from the penis is through the inguinal lymph nodes; thus, heterosexual men are affected most often in the inguinal lymph nodes.
- Homosexual men and women who are receptive to anal sex may develop perirectal and pelvic lymph node involvement. In women, these nodes may also become involved as a result of lymphatic spread from the cervix and posterior vaginal wall.
- Early in the course of the disease, the nodes appear fleshy and show diffuse reticulosis.
- Later, suppurative granulomatous lymphadenitis and perilymphadenitis occur with matting of the nodes. Frequently, these nodes coalesce to form stellate abscesses.
- Histologically, these abscesses are nearly diagnostic, but the clinical appearance may be similar to those seen in other infections, including catscratch fever and mycobacterial granulomatous infections.
The tertiary stage of LGV occurs years after the initial infection. In this stage, an anogenitorectal syndrome may occur with resultant rectal stricture or elephantiasis of the genitalia.
- This syndrome is found predominantly in women and homosexual men, because of the location of the involved lymphatics.
- This late stage is characterized by proctocolitis, which is caused by hyperplasia of intestinal and perirectal lymphatic tissue.
- This inflammation forms perirectal abscesses, ischiorectal abscesses, rectovaginal fistulas, anal fistulas, and rectal stricture. In very late stages, fibrosis and granulomas are characteristic.
- Chlamydial organisms are scarce at this stage.
Extragenital inoculation sites can produce regional lymphadenopathy. Examples are of mediastinal lymphadenopathy from inhalation of C trachomatis, or submandibular and cervical chain lymphadenopathy following inoculation after oral sex.
Frequency
United States
Sporadic cases occur in North America, Australia, and most of Asia. Most cases in the United States involve recent travel to an endemic area where the patient was sexually active; therefore, obtaining a travel history is important. Historically, the average number of LGV cases in the United States has been fewer than 600 per year.
International
LGV is endemic in East and West Africa, India, Southeast Asia, South America, the Caribbean, and Australia.1 Since 2003, an increasing epidemic of LGV with the L2 serovar has occurred in men who have sex with men in western Europe.
Mortality/Morbidity
- Given appropriate treatment, patients usually have complete resolution of symptoms.
- Death can occur from tertiary LGV if complete bowel obstruction from rectal stricture leads to perforation; this is rare, however.
Sex
LGV is diagnosed in men up to 6 times more frequently than in women.
Age
LGV infection is most common in the second and third decades when sexual activity is highest.
Clinical
History
- Primary lymphogranuloma venereum (LGV)
- The primary lesion of LGV occurs after an incubation period of 3-21 days following an exposure.
- The initial lesion may be a painless papule, shallow erosion, ulcer, or grouping of lesions with a herpetiform appearance.
- If the primary lesion is in the urethra, symptoms of a nonspecific urethritis may occur.
- The most common sites of primary infection in men include the coronal sulcus, frenulum, prepuce, penis, urethra, glans, and scrotum.
- In women, the most common sites of the primary lesion include the posterior vaginal wall, fourchette, posterior lip of the cervix, and vulva.
- The primary lesion is noticed in one third of affected men but rarely is observed in affected women.
- Primary lesions of the mouth can result from oral sexual exposure.
- Secondary LGV
- The secondary stage of LGV occurs after a usual incubation period of 10-30 days, but it may be up to 6 months. This stage is characterized by the formation of enlarged, tender regional lymph nodes known as buboes.
- Patients may experience constitutional symptoms, which can include fever, headache, malaise, chills, nausea, vomiting, and arthralgias.
- Tertiary LGV
- This late stage is characterized by proctocolitis.
- Symptoms include anal pruritus, bloody mucopurulent rectal discharge, fever, rectal pain, tenesmus, constipation, pencil-thin stools, and weight loss.
Physical
Often, the diagnosis is considered initially on the basis of physical findings. Clinical findings of large fluctuant buboes or draining sinuses are suggestive of the diagnosis of LGV. The presence of rectal stricture and/or perineal deformity in a young woman is highly suggestive of LGV.
- Primary LGV
- The initial lesion may be a painless papule, shallow erosion, ulcer, or herpetiform grouping of lesions.
- A cordlike lymphangitis of the dorsal penis may develop in primary LGV. This may progress to the formation of a solitary, large, tender lymphoid nodule, or bubonulus. These bubonuli may rupture to form sinuses and/or fistulas.
- Secondary LGV
- Buboes, which are enlarged, tender regional lymph nodes, may be present.
- The location of lymph node involvement is related directly to the site of the primary lesion.
- Inguinal lymphadenopathy occurs if the primary lesion involves the anterior vulva, penis, or urethra.
- Perirectal and pelvic lymphadenopathy result if the primary lesion involves the posterior vulva, vagina, or anus.
- Lymphadenitis of the submaxillary and cervical glands occurs if the site of primary inoculation is the mouth.
- Seventy-five percent of all patients have deep iliac nodal involvement, which seldom suppurates.
- In the classic presentation of the heterosexual man with inguinal lymph node involvement, a groove depression (groove sign) overlying the inguinal ligament is noted. This is caused by proliferation of inguinal and femoral lymph nodes, which are separated by the inguinal (Poupart) ligament. However, this presentation is seen in only 20% of affected men.
- Two thirds of patients with inguinal involvement have unilateral inguinal bubo formation with edema and erythema of the overlying skin.
- Frequently these nodes coalesce to form stellate abscesses.
- One third of these abscesses rupture; two thirds involute. Prior to a rupture, the skin overlying the buboes may become a dark livid color. After a rupture, pain decreases; however, a discharge may continue for weeks to months with the formation of a fistula or sinus tract.
- Cutaneous manifestations may accompany infection, including erythema multiforme, scarlatiniform eruption, urticaria, and, in 10% of cases, erythema nodosum.
- Complications of LGV that may be noted on physical examination include arthritis, conjunctivitis, and hepatomegaly. Pericarditis, pneumonia, and meningoencephalitis rarely occur.
- Tertiary LGV
- This stage is characterized by proctocolitis.
- Lymphorrhoids or perianal condylomata may be observed on examination of the rectum. These structures appear similar to hemorrhoids and are the result of an obstruction of lymphatics. They are composed of dilated lymph vessels with perilymphatic inflammation.
- Rectal examination at this stage also may reveal a granular mucosa and palpable, enlarged lymph nodes under the bowel wall. Stricture usually occurs 2-5 cm above the anocutaneous margin, and digital examination above the stricture may reveal smooth healthy mucosa.
- In very late stages, fibrosis and granulomas are characteristic.
- In women, esthiomene (eating away) occurs, which results in hypertrophic, chronic granulomatous enlargement of the vulva and subsequent ulceration. This may not appear for 1-20 years after the primary infection.
- In men, elephantiasis of the genitalia can occur.
Causes
- The causal organism is C trachomatis, serovars L1, L2, and L3.
- Serovar L2 is the most common cause.
- Risk factors
- Unprotected sex
- Anal intercourse
- Residing in or visiting tropical/developing countries
- Prostitution
More on Lymphogranuloma Venereum |
 Overview: Lymphogranuloma Venereum |
| Differential Diagnoses & Workup: Lymphogranuloma Venereum |
| Treatment & Medication: Lymphogranuloma Venereum |
| Follow-up: Lymphogranuloma Venereum |
| References |
| Further Reading |
| Next Page » |
References
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Further Reading
Clinical guidelines
New York State Department of Health. Lymphogranuloma venereum (LVG). New York (NY): New York State Department of Health; 2007 Aug. 11 p.
Clinical Effectiveness Group, British Association for Sexual Health and HIV (BASHH). National guideline for the management of lymphogranuloma venereum (LVG). London (UK): British Association for Sexual Health and HIV (BASHH); 2006. 14 p.
Herring A, Richens J, LGV Incident Group, Health Protection Agency. Lymphogranuloma venereum (LGV). In: Ross J, Ison C, Carder C, Lewis D, Mercey D, Young H. Sexually transmitted infections: UK national screening and testing guidelines. London (UK): British Association for Sexual Health and HIV (BASHH); 2006 Aug. p. 57-62.
U.S. Preventive Services Task Force. Behavioral counseling to prevent sexually transmitted infections: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med 2008 Oct 7;149(7):491-6, W95. 3
Keywords
lymphogranuloma venereum, lymphopathia venerea, tropical bubo, climatic bubo, strumous bubo, poradenitis inguinales, Durand-Nicolas-Favre disease, lymphogranuloma inguinale, LGV, sexually transmitted disease, STD, Chlamydia trachomatis, C trachomatis
