Lymphogranuloma venereum
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Lymphogranuloma venereum (LGV), also known as lymphopathia venerea, tropical bubo, climatic bubo, strumous bubo, poradenitis inguinales, Durand-Nicolas-Favre disease and lymphogranuloma inguinale, is a sexually transmitted disease caused by the invasive serovars L1, L2, or L3 of Chlamydia trachomatis. LGV was first described by Wallace in 1833 and again by Durand, Nicolas, and Favre in 1913.
LGV is primarily an infection of lymphatics and lymph nodes. Chlamydia trachomatis is the bacteria responsible for LGV. It gains entrance through breaks in the skin, or it can cross the epithelial cell layer of mucous membranes. The organism travels from the site of inoculation down the lymphatic channels to multiply within mononuclear phagocytes of the lymph nodes it passes.
In the United States, Europe, Australia and most of Asia and South America LGV is generally considered to be a rare disease. Only 42 cases were reported in the US in 2000. The incidence of LGV is on the rise since the early 2000's in homosexual populations. Example, the Netherlands, which typically has fewer than five cases a year, as of September 2004, a total of 92 cases of LGV had been confirmed during the preceding 17 months among men having sex with men. According to the New York State Department of Health, the incidence of LGV is highest among sexually active people living in tropical or subtropical climates, including some areas of the southern U.S. LGV is considered endemic in East and West Africa, India, parts of southeast Asia, South America, and the Caribbean.
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Signs and symptoms
Primary stage
LGV may begin as a self-limited painless genital ulcer that occurs at the contact site 3-12 days or longer in this primary stage. Rarely do women notice a primary infection, their inflamed lymph nodes are often deeper (because their infection site and drainage sites are usually different) and not as noticeable, and fewer that 1/3 of men notice as well. This primary stage heals in a few days.
Erythema nodosum occurs in 10% of cases.
Secondary stage
The secondary stage occurs from 10-30 days later most often, but has occurred up to 6 months later. The infection is then spread to the lymph nodes through lymphatic drainage pathways. The most frequent presenting clinical manifestation of LGV among males whose primary exposure was genital is unilateral, in 2/3 of cases, lymphadenitis and lymphangitis, often tender inguinal and/or femoral lymphadenopathy because of the drainage pathway for their likely infected areas. Lymphangitis of the dorsal penis may also occur and resembles string or cord. If the route was anal sex or contamination occurred via the fecal-oral route the infected person may experience lymphadenitis and lymphangitis noted above or may have proctitis, inflammation limited to the rectum (the distal 10--12 cm) that may be associated with anorectal pain, tenesmus, or rectal discharge, or proctocolitis, inflammation of the colonic mucosa extending to 12 cm above the anus and is associated with symptoms of proctitis plus diarrhea or abdominal cramps and or inflammatory involvement of perirectal or perianal lymphatic tissues. In females cervicitis, perimetritis, or salpingitis may occur as well as the lymphangitis and lymphadenitis in deeper nodes. Because of lymphatic drainage pathways, some end up with an abdominal mass which seldom suppurates and only 20-30% end up with inguinal lymphadenopathy. Systemic signs: fever, decreased appetite, and malaise, may occur as well. Diagnosis is more difficult in women and homosexual men who may not have the inguinal symptoms.
Over the course of the disease, lymph nodes enlarge, enlarged nodes are called buboes, and become painful at first (which may occur in any infection of the same areas as well). The next most common thing is inflammation, thinning and fixation of the overlying skin. Lastly in the progression are necrosis, fluctuant and suppurative lymph nodes, abscesses, fistulas, strictures, and sinus tracts all may occur. During the infection and when it subsides and healing takes place, fibrosis may occur. This can result in varying degrees of lymphatic obstruction, chronic edema, and strictures.
Prognosis
Highly variable. Spontaneous remission is common. Complete cure can be obtained with proper antibiotic treatment. Course is more favorable with early treatment. Bacterial superinfections may complicate course. Death can occur from bowel obstruction or perforation. Follicular conjunctivitis due to autoinoculation of infectious discharge.
Long term complications
Genital elephantiasis or esthiomene ,which is the dramatic end-result of lymphatic obstruction, which may occur because of the strictures themselves, or fistulas. This is usually seen if females, may ulcerate and often occurs 1-20 years after primary infection. Fistulas of, but not limited to, the penis, urethra, vagina, uterus, or rectum. Also, surrounding edema often occurs. Rectal or other strictures and scarring. Systemic spread may occur, possible results are arthritis, pneumonitis, hepatitis, or perihepatitis.
Diagnosis
The diagnosis usually is made serologically (through complement fixation) and by exclusion of other causes of inguinal lymphadenopathy or genital ulcers. Serologic testing is has a sensitivity of 80% after 2 weeks. Serologic testing may not be specific for serotype (has some cross reactivity with other chlamydia species) and can suggest LGV from other forms because of their difference in dilution, 1:64 more likely to be LGV and lower than 1:16 is likely to be other chlamydia forms (emedicine). For idenification of serotypes, culture is often used. Culture is difficult. Requiring a special media, cycloheximide-treated McCoy or HeLa cells, and yields are still only 30-50%. DFA, or direct fluorescent antibody test, PCR of likely infected areas and pus, are also sometimes used. DFA test for the L-type serovar of C trachomatis is the most sensitive and specific test, but is not readily available. If polymerase chain reaction (PCR) tests on infected material are positive, subsequent restriction endonuclease pattern analysis of the amplified outer membrane protein A gene can be done to determine the genotype. It has been noted that one type of testing my not be thorough enough.
Further recommendations
As with all STD's sex partners of patients who have LGV should be examined and tested for urethral or cervical chlamydial infection. After a positive culture fro chalamydia, clinical suspicion should be confirmed with testing to distinguish serotype. Antibiotic treatment should be started if they had sexual contact with the patient during the 30 days preceding onset of symptoms in the patient.
Patients with a sexually transmited disease need to be tested for other STD's. Antibiotics are not without risks and prophylaxtic broad antibiotic coverage is not recommended.
Treatment
Treatment involves Antibiotics and may involve drainage of the buboes or abscesses by needle aspiration or incision. Further supportive measure may need to be taken: dilatation of the rectal stricture, repair of rectovaginal fistulae, or colostomy for rectal obstruction.
Commonly antibiotic treatments include: tetracycline, doxycycline (adults only 2nd to teeth discoloration in children) and erythromycin.
References and external links
- Original article from the public domain resource "1998 Guidelines for Treatment of Sexually Transmitted Diseases" MMWR 47(RR-1);1-118 Publication date: 01/23/1998 at http://wonder.cdc.gov/wonder/prevguid/p0000480/p0000480.asp#head007005000000000 note that this has not been modified since 1998, and may be out of date.
- Article on eMedicine.com (http://www.emedicine.com/EMERG/topic304.htm) Last Updated: 16 October 2004
- Centers for Disease Control and Prevention. Proctitis, proctocolitis, and enteritis. Sexually transmitted diseases treatment guidelines. MMWR Recomm Rep 2002 10 May;51(RR-6):66-7.
- Fitzpatrick's color atlas and synopsis of clinical dermatology 5th ed. Wolff et al.
- Rosen T. Brown: Genital ulcers - evaluation and treatment. Dermatology Clin. 1998;16:673.
- Nederlands tijdschrift voor geneeskunde. vol. 148, no. 51 (2004 18 December): 2544-6.
- Dermatology. vol. 209, no. 3 (2004): 230-2.
- MMWR. Morbidity and mortality weekly report. vol. 53, no. 42 (2004 29 October): 985-8.
- International journal of STD & AIDS. vol. 13, no. 6 (2002 Jun): 427-9.
- http://www.essti.org/