Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Pathophysiology | Diagnosis | Case reports | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Rishi A. Lymphogranuloma venereum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/anusLGV.html. Accessed December 4th, 2024.
Definition / general
- Sexually transmitted chronic ulcerative disease
- Causative organism Chlamydia trachomatis L1, L2, and L3 serovars
- Complications: strictures, perirectal abscess, fistulas and sinuses, squamous cell carcinoma
- Rectal strictures are more common in women
Terminology
- Three stages of infection:
- Primary infection:
- Small often unnoticed ulceration, erosion or erythematic area
- Usually self healing
- Incubation period of 3 - 12 days following an exposure to the causative organism
- Secondary infection:
- Due to the extension of infection to the draining lymph nodes
- LGV causing serovars produce severe disease with systemic symptoms as compared to serovars A to K
- Manifests 2 - 6 weeks after primary infection
- Characterized by enlarged and painful lymph nodes
- Lymph nodes may rupture and form cutaneous sinuses
- Anorectal disease presents as GI bleed, discharge, rectal ulcer, anal pain or constipation
- Clinically and histologically mimics inflammatory bowel disease
- Late infection:
- Complications of longstanding disease in the form of strictures, fistulas, genital lymphedema and infertility
- Primary infection:
Epidemiology
- Endemic disease in the tropical and subtropical regions of Asia, Africa, South America and Africa
- Increased incidence in western population, especially men who have sex with men (MSM)
- Associated coinfections:
- Up to 76% also infected with HIV (Clin Infect Dis 2007;44:26)
- Other sexually transmitted disease in 39%
- Hepatitis C infection in 20%
Sites
- Rectum (LGV proctitis)
- Perianal skin
- Draining lymph nodes
Pathophysiology
- Mainly involves lymphatic system causing lymphangitis, necrosis and abscess formation
- Produces more generalized lymphoproliferative response
- Binds through heparan sulfate receptors
- Recombination of serovars had been reported between serovars L2 and D in cases with hemorrhagic proctitis (MBio 2011;2:e00045)
Diagnosis
- Culture of the organism
- Sampling by direct aspirate from the infected lymph node, perianal swab or rectal swab
- Positive culture is confirmatory but the yield is usually low
- Culture is positive in 20 - 30% of cases and depends on the stage of the illness
- Most common positive culture is during the secondary stage of illness
- Primary stage is usually missed for sampling because of subtle clinical feature
- Chlamydia cultures are not widely available and are done only at referral centers
- Serology
- Complement fixation test and immunofluorescence test
- Complement fixation test measures antibody against group specific lipopolysaccharide antigen
- Serological tests are supportive only in the presence of appropriate clinical findings
- Rising titer is significant for the presence of the disease
- Titer of greater than 1:64 is considered supportive
- Limitation:
- Cannot distinguish recent from past infection
- Cannot distinguish individual serotypes
- Limited diagnostic potential in an isolated site infection
- Nucleic acid amplification test (NAAT)
- A polymerase chain reaction (PCR) based test
- Sensitivity and specificity greater than 95%
- Ease of sampling (swab) and transport of the specimen
- Routine additional testing following a positive NAAT screening test for C. trachomatis no longer recommended by CDC
- See details of CDC recommendation for laboratory diagnosis at MMWR Recomm Rep 2014;63:1
Case reports
- 17 year old boy with LGV and non-Hodgkin lymphoma (Rev Soc Bras Med Trop 2012;45:412)
- 28, 30 and 42 year old men with LGV proctosigmoiditis mimicking inflammatory bowel disease (World J Gastroenterol 2012;18:3317)
- 60 year old man with squamous cell carcinoma of penis with bullous pemphigoid masquerading as LGV (Indian J Sex Transm Dis 2013;34:41)
- May present as proctitis (Rev Esp Enferm Dig 2014;106:59)
Microscopic (histologic) description
- Granulomatous proctitis resembling Crohn's disease
- Follicular infiltrates of lymphocytes, histiocytes and plasma cells, neural hyperplasia, extensive fibrosis
- See also Lymph nodes - Lymphogranuloma venereum
Microscopic (histologic) images
Differential diagnosis
- Crohn's disease
- Other infections causing stricture and fistulas: tuberculosis, fungal infections
- Squamous cell carcinoma