eMedicine Specialties > Dermatology > Bacterial Infections

Lymphogranuloma Venereum

Author: Jose Antonio Plaza, MD, Director of Dermatopathology, Department of Pathology, Froedtert Hospital; Assistant Professor, Department of Pathology, Section of Dermatopathology, Medical College of Wisconsin
Coauthor(s): Victor G Prieto, MD, PhD, Director of Dermatopathology, Professor, Departments of Pathology and Dermatology, University of Texas MD Anderson Cancer Center
Contributor Information and Disclosures

Updated: Sep 2, 2009

Introduction

Background

Lymphogranuloma venereum (LGV) is a primarily cutaneous, and sometimes systemic, sexually transmitted disease (STD), which primarily affects lymphatic tissue of the groin. LGV is caused by unique serotypes L1, L2, and L3 of Chlamydia trachomatis. LGV occurs only sporadically in North America, but it is endemic in many parts of the developing world. A recent outbreak of LGV proctocolitis has been reported among homosexual men in North America and Europe, and many of these individuals were co-infected with HIV.1,2,3,4,5

Pathophysiology

Lymphogranuloma venereum (LGV) is caused by C trachomatis, an obligate intracellular pathogen (ie, the bacterium lives within human cells), and strains L1, L2, and L3 have been associated with infection. LGV is primarily a disease of lymphatic tissue. Because Chlamydia species cannot traverse the intact epithelial barrier, access to lymphatic vessels is gained through microtrauma in the skin or mucous membranes. The pathogen then enters the draining lymph nodes, causing lymphangitis or lymphadenitis. The causal pathologic process involves thrombolymphangitis and perilymphangitis and the consequent spread of the inflammatory reaction from the affected lymph nodes to surrounding tissues.

Frequency

United States

Lymphogranuloma venereum (LGV) is rare in the United States, and the true incidence is not known.

International

Lymphogranuloma venereum (LGV) is most common in Southeast Asia, Africa, Central America, and the Caribbean. LGV accounts for 2-10% of genital ulcer disease in India and Africa.

Mortality/Morbidity

Progression to the third phase of lymphogranuloma venereum (LGV) can result in serious and permanent sequelae such as genital deformity, fistulas, and rectal strictures, among others. Complete cure is achieved by early recognition of LGV and appropriate antibiotic treatment.

Race

Lymphogranuloma venereum (LGV) is found more commonly in blacks.

Sex

Lymphogranuloma venereum (LGV) is significantly more common in men than in women.

Age

The peak range for lymphogranuloma venereum (LGV) is in individuals aged 15-40 years.

Clinical

History

Clinical manifestations of lymphogranuloma venereum (LGV) vary depending on the sex of the patient, his or her sexual practices (vaginal or anal intercourse), and disease stage. Immunosuppression also seems to result in more severe or prolonged symptoms. LGV is a chronic and progressively destructive venereal disease and is divided into primary, secondary, and tertiary stages.

  • The primary stage of LGV is characterized by transient nonpainful papules, ulcers, or herpetiform erosions that may manifest after an incubation period of 3–12 days. The lesion usually remains unnoticed by the patient. Travel and sexual histories are important because LGV often is seen in people who have been sexually active in areas where the disease is endemic. Occasionally patients may have balanitis, balanoposthitis, cervicitis, salpingitis, or parametritis. The primary stage of disease is observed in less than 30% of heterosexual men and less frequently in women.
  • The symptoms seen in the secondary stage of LGV result from the spread of inflammation to regional lymphatic tissue. Depending on the entry site, inguinal lymphadenopathy is the classic manifestation of the secondary stage of LGV, and it occurs 2-6 weeks after the onset of primary symptoms. Patients tend to present with painful inguinal lymphadenopathy that usually is unilateral. In  one third of patients, the affected lymph node ruptures spontaneously after abscessing and developing areas of necrosis. Extragenital primary manifestations also involve regional lymph nodes with lymphadenopathy and the formation of buboes. Constitutional symptoms, such as fever, chills, malaise, myalgias, and arthralgias, are common in this stage of the disease. Systemic spread occasionally can result in arthritis, pneumonitis, or hepatitis.
  • Given the persistence of the pathogen, LGV reaches the tertiary stage in 25% of untreated patients. The chronic inflammatory reaction can lead to fistulas, strictures, rectal stenoses, and lymphedema. As a result of inguinal lymphadenopathy and chronic lymphedema with sclerosing fibrosis, elephantiasis may occur, affecting the penis or scrotum or the labia majora or clitoris. Additionally, other symptoms include fever, pain, tenesmus, pruritus, and purulent or bloody diarrhea.

Physical

  • Primary stage of lymphogranuloma venereum (LGV)
    • The primary lesion is a small painless papule or herpetiform ulcer on the genitalia.
    • The lesion usually heals within a few days; therefore, it is identified in only approximately 10% of patients at initial presentation.
    • When present, lesions are found most typically on the glans penis or vaginal wall.
  • Secondary stage of LGV
    • The most prominent physical finding at the secondary stage is unilateral painful inguinal lymphadenopathy.
    • A characteristic physical finding, termed the groove sign, occurs in approximately one third of patients. This sign is caused by enlargement of the nodes above and below the inguinal ligament.
    • One third of the inguinal buboes become fluctuant and rupture, while the remaining two thirds involute to form a hard nonsuppurative inguinal mass.
    • A 10:1 predominance of buboes exists in men compared with women who reach this stage of disease.
    • Women often have primary involvement of the rectum, vagina, cervix, or posterior urethra, which drain to the deep iliac or perirectal nodes; therefore, only 20-30% have the classic finding of inguinal lymphadenopathy.
  • Tertiary stage of LGV
    • Physical findings at the tertiary stage include proctocolitis, perirectal abscess, fistulas, strictures, and hyperplasia of the intestinal and perirectal lymphatics (lymphorrhoids).
    • Chronic infection can result in extensive scarring with ischemia and tissue necrosis.
    • The end result can be esthiomene (elephantiasis of the female genitalia characterized by fibrotic labial thickening) in women or elephantiasis and deformation of the penis in men.

Causes

  • The causal organism of lymphogranuloma venereum (LGV) is C trachomatis, serotypes L1, L2, and L3; L2 is the most common.
  • Risk factors include the following:
    • Visiting endemic areas
    • Engaging in unprotected sex
    • Engaging in anal intercourse
    • History of multiple sex partners

More on Lymphogranuloma Venereum

Overview: Lymphogranuloma Venereum
Differential Diagnoses & Workup: Lymphogranuloma Venereum
Treatment & Medication: Lymphogranuloma Venereum
Follow-up: Lymphogranuloma Venereum
References
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References

  1. Bremer V, Meyer T, Marcus U, Hamouda O. Lymphogranuloma venereum emerging in men who have sex with men in Germany. Euro Surveill. Sep 2006;11(9):152-4. [Medline].

  2. Herida M, de Barbeyrac B, Sednaoui P, et al. Rectal lymphogranuloma venereum surveillance in France 2004-2005. Euro Surveill. Sep 2006;11(9):155-6. [Medline].

  3. Kapoor S. Re-emergence of lymphogranuloma venereum. J Eur Acad Dermatol Venereol. Apr 2008;22(4):409-16. [Medline].

  4. Klint M, Lofdahl M, Ek C, Airell A, Berglund T, Herrmann B. Lymphogranuloma venereum prevalence in Sweden among men who have sex with men and characterization of Chlamydia trachomatis ompA genotypes. J Clin Microbiol. Nov 2006;44(11):4066-71. [Medline].

  5. van de Laar MJ. The emergence of LGV in western Europe: what do we know, what can we do?. Euro Surveill. Sep 2006;11(9):146-8. [Medline].

  6. Chen CY, Chi KH, Alexander S, et al. The molecular diagnosis of lymphogranuloma venereum: evaluation of a real-time multiplex polymerase chain reaction test using rectal and urethral specimens. Sex Transm Dis. Jul 2007;34(7):451-5. [Medline].

  7. Hadfield TL, Lamy Y, Wear DJ. Demonstration of Chlamydia trachomatis in inguinal lymphadenitis of lymphogranuloma venereum: a light microscopy, electron microscopy and polymerase chain reaction study. Mod Pathol. Dec 1995;8(9):924-9. [Medline].

  8. Klotz SA, Drutz DJ, Tam MR, Reed KH. Hemorrhagic proctitis due to lymphogranuloma venereum serogroup L2. Diagnosis by fluorescent monoclonal antibody. N Engl J Med. Jun 30 1983;308(26):1563-5. [Medline].

  9. Gilleece Y, Sullivan A. Management of sexually transmitted infections in HIV positive individuals. Curr Opin Infect Dis. Feb 2005;18(1):43-7. [Medline].

  10. [Guideline] Lymphogranuloma venereum (LVG). New York State Department of Health. National Guidelines Clearinghouse. Aug 2007.

  11. [Guideline] U.S. Preventive Services Task Force. Behavioral counseling to prevent sexually transmitted infections: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. Oct 7 2008;149(7):491-6, W95. [Medline].

  12. Blank S, Schillinger JA, Harbatkin D. Lymphogranuloma venereum in the industrialised world. Lancet. May 7-13 2005;365(9471):1607-8. [Medline].

  13. Brown TJ, Yen-Moore A, Tyring SK. An overview of sexually transmitted diseases. Part I. J Am Acad Dermatol. Oct 1999;41(4):511-32. [Medline].

  14. Burgoyne RA. Lymphogranuloma venereum. Prim Care. Mar 1990;17(1):153-7. [Medline].

  15. Den Hollander JG, Ossewaarde JM, de Marie S. Anorectal ulcer in HIV patients, don't forget lymphogranuloma venereum!. AIDS. Jul 2 2004;18(10):1484-5. [Medline].

  16. Joseph AK, Rosen T. Laboratory techniques used in the diagnosis of chancroid, granuloma inguinale, and lymphogranuloma venereum. Dermatol Clin. Jan 1994;12(1):1-8. [Medline].

  17. Kellock DJ, Barlow R, Suvarna SK, Green S, Eley A, Rogstad KE. Lymphogranuloma venereum: biopsy, serology, and molecular biology. Genitourin Med. Oct 1997;73(5):399-401. [Medline].

  18. Martin DH, Mroczkowski TF. Dermatologic manifestations of sexually transmitted diseases other than HIV. Infect Dis Clin North Am. Sep 1994;8(3):533-82. [Medline].

  19. Martin IM, Alexander SA, Ison CA, Macdonald N, McCarthy K, Ward H. Diagnosis of lymphogranuloma venereum from biopsy samples. Gut. Oct 2006;55(10):1522-3. [Medline].

  20. McLean CA, Stoner BP, Workowski KA. Treatment of Lymphogranuloma Venereum. Clinical Infectious Diseases. 2007;44:S147–S152.

  21. Papagrigoriadis S, Rennie JA. Lymphogranuloma venereum as a cause of rectal strictures. Postgrad Med J. Mar 1998;74(869):168-9. [Medline].

  22. Rosen T, Brown TJ. Cutaneous manifestations of sexually transmitted diseases. Med Clin North Am. Sep 1998;82(5):1081-104, vi. [Medline].

  23. Simms I, Ward H, Martin I, Alexander S, Ison C. Lymphogranuloma venereum in Australia. Sex Health. Sep 2006;3(3):131-3. [Medline].

  24. Sternberg SJ. The penis. Diagn Surg Pathol. 1999;2(3):2039-40.

  25. Sturm PD, Moodley P, Govender K, Bohlken L, Vanmali T, Sturm AW. Molecular diagnosis of lymphogranuloma venereum in patients with genital ulcer disease. J Clin Microbiol. Jun 2005;43(6):2973-5. [Medline].

  26. Trager JD. Sexually transmitted diseases causing genital lesions in adolescents. Adolesc Med Clin. Jun 2004;15(2):323-52. [Medline].

  27. Van Vranken M. Prevention and treatment of sexually transmitted diseases: an update. Am Fam Physician. Dec 2007;15;76(12):1827-32.

  28. Weir E. Lymphogranuloma venereum in the differential diagnosis of proctitis. CMAJ. Jan 18 2005;172(2):185. [Medline].

  29. White J, Ison C. Lymphogranuloma venereum: what does the clinician need to know?. Clin Med. Jun 2008;8(3):327-30. [Medline].

  30. [Guideline] Workowski KA, Berman SM, Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines, 2006. MMWR Recomm Rep. Aug 4 2006;55(RR-11):1-94. [Medline].

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Further Reading

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Keywords

lymphogranuloma venereum, LGV, Chlamydia trachomatis, C trachomatis, STD, sexually transmitted chlamydial disease, sexually transmitted disease, chlamydia

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Contributor Information and Disclosures

Author

Jose Antonio Plaza, MD, Director of Dermatopathology, Department of Pathology, Froedtert Hospital; Assistant Professor, Department of Pathology, Section of Dermatopathology, Medical College of Wisconsin
Jose Antonio Plaza, MD is a member of the following medical societies: American Medical Association and American Society for Clinical Pathology
Disclosure: Nothing to disclose.

Coauthor(s)

Victor G Prieto, MD, PhD, Director of Dermatopathology, Professor, Departments of Pathology and Dermatology, University of Texas MD Anderson Cancer Center
Victor G Prieto, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Medical Association, American Society for Clinical Pathology, American Society of Dermatopathology, College of American Pathologists, European Society of Pathology, International Society of Dermatopathology, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Medical Editor

Terry L Barrett, MD, Clinical Professor of Dermatology and Pathology, University of Texas Southwestern School of Medicine; Director, ProPath Dermatopathology, Dallas, Texas
Terry L Barrett, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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