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| {{Infobox_Disease |
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| Name = {{PAGENAME}} |
| | '''For patient information, click [[Lymphogranuloma venereum (patient information)|here]]''' |
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| Caption = |
| | {{Lymphogranuloma venereum}} |
| DiseasesDB = 29101 |
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| ICD10 = {{ICD10|A|55||a|50}} |
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| ICD9 = {{ICD9|099.1}} |
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| ICDO = |
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| OMIM = |
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| MedlinePlus = 000634 |
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| eMedicineSubj = emerg |
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| eMedicineTopic = 304 |
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| eMedicine_mult = {{eMedicine2|med|1356}} {{eMedicine2|derm|617}} |
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| MeshID = D008219 |
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| {{SI}} | |
| {{CMG}} | | {{CMG}} |
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| {{Editor Help}} | | {{SK}} Durand-Nicolas-Favre disease; Nicolas-Favre disease; LGV; lymphogranuloma inguinale; lymphopathia venereum; lymphopathia venerea; poradenitis inguinale; poradentitis; tropical bubo; climatic bubo; strumous bubo; sixth venereal disease |
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| ==Overview==
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| '''Lymphogranuloma venereum''' ('''LGV'''), also known as lymphopathia venerea, tropical bubo, climatic bubo, strumous bubo, poradenitis inguinales, Durand-Nicolas-Favre disease, lymphogranuloma inguinale and neekerisankkeri in Finland, is a [[sexually transmitted disease]] caused by the invasive [[Biovar|serovars]] L1, L2, or L3 of ''[[Chlamydia trachomatis]]''.
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| LGV was first described by Wallace in 1833<ref name="EmedicineEmerg304">{{cite web |url=http://www.emedicine.com/EMERG/topic304.htm |title=eMedicine - Lymphogranuloma Venereum : Article by Andrew C Bushnell |accessdate=2007-10-26 |format= |work=}}</ref> and again by Durand, Nicolas, and Favre in 1913. <ref>{{WhoNamedIt|synd|1431}}</ref><ref>N. J. Durand, J. Nicolas, M. Favre. Lymphogranulomatose inguinale subaiguë d’origine génitale probable, peut-être vénérienne. Bulletin de la Société des Médecins des Hôpitaux de Paris, 1913, 3 sér., 35: 274-288. </ref>
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| ==Overview==
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| 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 membrane]]s. 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]]. However, a recent outbreak in the Netherlands among gay men has led to an increase of LGV in Europe and the United States.<ref>Thomas H. Maugh II. [http://www.latimes.com/news/printedition/asection/la-sci-lgv11may11,1,5266183.story?coll=la-news-a_section Virulent Chlamydia Detected Largely Among Gay Men in U.S.] Los Angeles Times: 11 May 2006</ref><ref> Michael Brown. [http://www.aidsmap.com/en/news/A50D26E2-D9AF-490F-B239-BD9D984645F2.asp LGV in the UK: almost 350 cases reported and still predominantly affecting HIV-positive gay men] Aidsmap: 17 May 2006</ref> A majority of these patients are HIV co-infected. Since the 2004 Dutch outbreak 341 cases have been reported in the UK and 80 cases in the US, but infectious-disease experts fear the actual number is substantially larger because this form of chlamydia is difficult to diagnose and many physicians are not aware of its existence.
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| Soon after the initial Dutch report national and international health authorities launched warning initiatives and multiple LGV cases where identified in several more European countries (Belgium, France, the UK, Germany, Sweden, Italy and Switzerland) and the US and Canada. All cases reported in Amsterdam and France and a considerable part of LGV infections in the UK and Germany are caused by a newly discovered Chlamydia variant L2b, a.k.a the Amsterdam variant. The L2b variant could be traced back and was isolated from anal swabs of men who have sex with men who visited the STI city clinic of San Francisco in 1981. This finding suggests that the recent LGV outbreak among MSM in industrialised countries is a slowly evolving epidemic. As of end 2005, new LGV cases are continued to be reported in the Netherlands and other European countries at rates approaching one or two cases per week in each country.
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| Picture of Lymphogranuloma venereum:
| | ==[[Lymphogranuloma venereum overview|Overview]]== |
| [http://members.shaw.ca/fartpipe/temp/crap/Bluesky.jpg]
| | ==[[Lymphogranuloma venereum historical perspective|Historical Perspective]]== |
| | | ==[[Lymphogranuloma venereum pathophysiology|Pathophysiology]]== |
| ==Signs and symptoms== | | ==[[Chlamydia trachomatis|Causes]]== |
| The clinical manifestation of LGV depends on the site of entry of the infectious organism (the sex contact site) and the stage of disease progression. Inoculation at the mucous lining of external sex organs (penis and vagina) can lead to the '''inguinal syndrome''' named after the formation of buboes or [[abscess]]es in the groin (inguinal) region where draining lymph nodes are located. The '''rectal syndrome''' arises if the infection takes place via the rectal mucosa (through anal sex) and is mainly characterized by [[proctocolitis]] symptoms. The '''pharyngeal syndrome''' is rare, starts after infection of pharyngeal tissue and buboes in the neck region can occur.
| | ==[[Lymphogranuloma venereum classification|Classification]]== |
| | | ==[[Lymphogranuloma venereum differential diagnosis|Differentiating Lymphogranuloma venereum from other Diseases]]== |
| ===Primary stage=== | | ==[[Lymphogranuloma venereum epidemiology and demographics|Epidemiology and Demographics]]== |
| 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, because the initial ulceration where the organism penetrates the mucosal layer are located out of sight in the vaginal wall. Also in men fewer than 1/3 of those infected notice the first signs of LGV. This primary stage heals in a few days. [[Erythema nodosum]] occurs in 10% of cases.
| | ==[[Lymphogranuloma venereum risk factors|Risk Factors]]== |
| | | ==[[Lymphogranuloma venereum natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| ===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, [[adenitis|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 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 tissue]]s. 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.
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| 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, [[abscess]]es, 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 [[stricture]]s. These late stages characterised by fibrosis and edema are also known as the third stage of LGV and are mainly permanent.
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| ==Prognosis==
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| 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.
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| ==Long term complications==
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| 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 in females, may ulcerate and often occurs 1-20 years after primary infection.
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| 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.
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| ==Diagnosis== | | ==Diagnosis== |
| The [[diagnosis]] usually is made [[Serology|serologically]] (through complement fixation) and by exclusion of other causes of inguinal lymphadenopathy or [[genital]] [[ulcer]]s. Serologic testing 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. Recently a fast realtime PCR (Taqman analysis) has been developed to diagnose LGV. With this method an accurate diagnosis is feasible within a day. It has been noted that one type of testing may not be thorough enough.
| | [[Lymphogranuloma venereum history and symptoms|History and Symptoms]] | [[Lymphogranuloma venereum physical examination|Physical Examination]] | [[Lymphogranuloma venereum laboratory findings|Laboratory Findings]] | [[Lymphogranuloma venereum other diagnostic studies|Other Diagnostic Studies]] |
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| ==Further recommendations==
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| As with all [[Sexually transmitted disease|STD]]'s sex partners of patients who have LGV should be examined and tested for [[urethra]]l or [[cervix|cervical]] [[chlamydia]]l infection. After a positive culture for chlamydia, 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 transmitted disease need to be tested for other STD's. Antibiotics are not without risks and prophylaxtic broad antibiotic coverage is not recommended.
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| ==Treatment== | | ==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.
| | [[Lymphogranuloma venereum medical therapy|Medical Therapy]] | [[Lymphogranuloma venereum surgery|Surgery]] | [[Lymphogranuloma venereum prevention|Prevention]] | [[Lymphogranuloma venereum cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Lymphogranuloma venereum future or investigational therapies|Future or Investigational Therapies]] |
| Common antibiotic treatments include: [[tetracycline]], [[doxycycline]] (all tetracyclines, including doxycycline, are contraindicated during pregnancy and in children due to effects on bone development and tooth discoloration), and [[erythromycin]].
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| ==References==
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| <references/>
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| ==Additional Resources==
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| * Original article from the public domain resource "1998 Guidelines for Treatment of Sexually Transmitted Diseases" MMWR 47(RR-1);1-118 Publication date: 23 January 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.
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| * 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.
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| * Fitzpatrick's color atlas and synopsis of clinical dermatology 5th ed. Wolff et al.
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| * Rosen T. Brown: Genital ulcers - evaluation and treatment. Dermatology Clin. 1998;16:673.
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| * Nederlands tijdschrift voor geneeskunde. vol. 148, no. 51 (18 December 2004): 2544-6.
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| * Dermatology. vol. 209, no. 3 (2004): 230-2.
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| * MMWR. Morbidity and mortality weekly report. vol. 53, no. 42 (29 October]] 2004 ): 985-8.
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| * International journal of STD & AIDS. vol. 13, no. 6 (2002 Jun): 427-9.
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| * http://www.essti.org/
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| * Emerging Infectious Diseases vol. 11 no. 7 July 2005: 1090-2. [http://www.cdc.gov/ncidod/EID/vol11no07/04-0883.htm]
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| * Emerging Infectious Diseases vol. 11 no. 8 August 2005: 1311-2.
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| * Emerging Infectious Diseases vol. 11 no. 11 November 2005: 1787-8.
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| * Clinical Infectious Diseases vol. 42 no. 2 January 15 2006: 186-94.
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| | ==Case Studies== |
| | [[Lymphogranuloma venereum case study one|Case #1]] |
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| {{STD/STI}} | | {{STD/STI}} |
| {{Bacterial diseases}} | | {{Bacterial diseases}} |
| {{SIB}}
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| [[fi:Neekerisankkeri]] | | [[fi:Neekerisankkeri]] |
| [[sv:Lymphogranuloma venereum]] | | [[sv:Lymphogranuloma venereum]] |
| | [[Category:Bacterial diseases]] |
| | [[Category:Sexually transmitted diseases]] |
| | [[Category:Disease]] |
| | [[Category:Gynecology]] |
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| [[Category:Sexually transmitted diseases]]
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| [[Category:Infectious disease]]
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