Head and neck manifestations of lymphogranuloma venereum.Abstract
Lymphogranuloma venereum (LGV)--caused by Chlamydia trachomatis serovars L1, L2, or L3--rarely occurs in the United States. The disease clinically manifests in three stages:primary, secondary, and tertiary. The primary manifestation, a self-limited genital ulcer at the site of inoculation, often is absent by the time the patient seeks medical attention. The most common clinical manifestation of LGV is evident in its secondary stage: unilateral tender inguinal inguinal /in·gui·nal/ (in´gwi-n'l) pertaining to the groin.
1. Of or located in the groin.
2. and/or femoral lymphadenopathy. However, proctocolitis or inflammatory involvement of perirectal or perianal perianal
around the anus.
under the skin outside the anal canal. Causes sufficient pain to inhibit defecation. lymphatic tissues resulting in fistulas and strictures may also occur. The diagnosis of LGV is usually made serologically and by exclusion of other causes of inguinal lymphadenopathy or genital ulcers. Doxycydine is the preferred treatment; it cures the infection and prevents ongoing tissue damage. This case highlights an unusual manifestation of LGV infection--cervical lymphadenopathy following suspected oropharyngeal infection with C trachomatis. Head and neck manifestations of LGV may become an increasing problem in the future if sexual practices such as orogenital contact become more widespread.
Lymphogranuloma venereum (LGV) is a sexually transmitted disease sexually transmitted disease (STD) or venereal disease, term for infections acquired mainly through sexual contact. Five diseases were traditionally known as venereal diseases: gonorrhea, syphilis, and the less common granuloma inguinale, caused by Chlamydia trachomatis serovars L1, L2, or L3. (1) The disease clinically manifests in three stages: a primary lesion consisting of a superficial ulcer or erosion, a secondary stage with prominent lymphadenopathy and constitutional symptoms, and a later stage with disease manifestations such as fibrosis, lymphedema, and anorectal a·no·rec·tal
Relating to the anus and the rectum.
pertaining to, emanating from or affecting the anorectum.
see perianal fistula. strictures. (1,2)
The diagnosis of LGV is most commonly made by a combination of tests, including biopsy of the involved lymph node and serologic tests (e.g., complement fixation [CF] or microimmunofluorescence [MIF] tests). (3-5) Laboratory criteria consistent with a diagnosis of LGV include a C trachomatis IgG titer >1:64 on a CF test or a titer >1:128 on an MIF test. (5) In addition to the above tests, direct detection of the organism by nucleic acid amplification tests (NAATs) on a swabbed specimen of the affected area would definitively confirm the diagnosis. (5) However, the presence of a characteristic clinical syndrome in combination with a positive serologic test is considered adequate for a presumptive diagnosis of LGV. (1)
Although LGV characteristically presents as inguinal adenopathy, rare cases of LGV involving the cervical lymph nodes have been recognized. (6) In this report, we describe a patient in whom cervical lymphadenopathy, with probable oropharyngeal involvement, was the presenting manifestation of LGV.
An 18-year-old woman was admitted with symptoms of fever and cervical lymphadenopathy. Three days before admission, she had noticed an enlarging, tender lymph node on the left side of her neck and had subsequently developed fever, chills, and generalized body weakness. She denied a recent history of sore throat, rash, or inguinal adenopathy and stated that she had had no recent sexual contacts. The patient's medical history was noncontributory, and she denied human immunodeficiency virus human immunodeficiency virus
Human immunodeficiency virus (HIV)
A transmissible retrovirus that causes AIDS in humans. (HIV) risk factors, a history of sexually transmitted disease, drug use, or exposure to animals or sick human contacts.
On admission, her oral temperature was 40.1[degrees]C and the rest of her vital signs were within normal limits. The oral cavity appeared normal; however, indirect laryngoscopy revealed mild hypopharyngeal erythema with hyperplasia of the adenoidal ad·e·noi·dal
1. Of or relating to the adenoids.
2. Suggestive of the vocal sound caused by abnormally enlarged adenoids: a singer with an adenoidal voice. bed. Examination of the neck showed a tender, palpable, nonfluctuant 3 x 3-cm lymph node in the left posterior triangle of the neck The posterior triangle (or lateral cervical region) is a region of the neck. Boundaries
It has the following boundaries:
The patient's white blood cell count white blood cell count,
n a diagnostic clinical laboratory test to determine the number and types of leukocytes present in a measured sample of blood. Overall the normal number of leukocytes ranges from 5000 to 10,000/mm3. was 6,100 cells/ [mm.sup.3 with a normal differential cell count. The erythrocyte sedimentation rate Erythrocyte Sedimentation Rate Definition
The erythrocyte sedimentation rate (ESR), or sedimentation rate (sed rate), is a measure of the settling of red blood cells in a tube of blood during one hour. was elevated at 47 mm/hr. Urinalysis, electrolytes, and liver function studies were all within normal limits. A chest roentgenogram roent·gen·o·gram
A photograph made with x-rays. Also called roentgenograph.
roentgenogram (rent´g was normal; however, an abdominal ultrasound showed splenomegaly splenomegaly /sple·no·meg·a·ly/ (-meg´ah-le) enlargement of the spleen.
congestive splenomegaly Banti's disease; splenomegaly secondary to portal hypertension. .
The admitting diagnosis was cervicallymphadenitis--most likely caused by Staphylococcus aureus, group A Streptococcus group A streptococcus
A common but virulent streptococcus that kills the tissue it infects and produces toxins that trigger a form of shock that affects the vital organs. , or mixed aerobic-anaerobic bacterial infection--and the patient was started on IV ampicillin/sulbactam. Additional considerations included lymphadenitis Lymphadenitis Definition
Lymphadenitis is the inflammation of a lymph node. It is often a complication of a bacterial infection of a wound, although it can also be caused by viruses or other disease agents. secondary to Epstein-Barr virus (EBV) infection, syphilis, HIV infection, mycobacterial infection, coccidioidomycosis coccidioidomycosis (kŏksĭd'ēoi'dōmīkō`sĭs), systemic fungus disease (see fungal infection) endemic to arid regions of the Americas, contracted by inhaling dust containing spores of the fungus Coccidioides immitis. , histoplasmosis histoplasmosis: see fungal infection. , cat-scratch disease, and sarcoidosis Sarcoidosis Definition
Sarcoidosis is a disease which can affect many organs within the body. It causes the development of granulomas. Granulomas are masses resembling little tumors. They are made up of clumps of cells from the immune system. .
Despite 10 days of IV antibiotic therapy, the patient's fever persisted and the cervical lymph node remained enlarged and tender. Urine and blood cultures, obtained upon admission and during hospitalization, remained negative. Serologies for syphilis (rapid plasma reagin), HIV, EBV, Coccidoides immitis, and Bartonella henselae were negative. The patient had a negative test for purified protein derivative purified protein derivative
see purified protein derivative of tuberculin. . Her serum angiotensin-converting enzyme level was normal, and she tested negative for urine histoplasma antigen.
Cervical computed tomography (CT) demonstrated prominent soft-tissue swelling within Waldeyer's ring and bilateral cervical lymphadenopathy with a large, necrotic left cervical lymph node possibly containing fluid; there was no evidence of a retropharyngeal abscess. Because of the patient's failure to respond to standard antibacterial therapy, she underwent fine-needle aspiration of the left posterosuperior cervical lymph node. This showed reactive lymphoid hyperplasia and findings consistent with granulomatous inflammation. The acidfast stain for mycobacteria and the Gomori methenamine methenamine /meth·en·amine/ (meth?en-am´in) an antibacterial used in urinary tract infections; administered as the hippurate and mandelate salts.
n. silver stain were negative.
On more careful questioning, the patient stated that several weeks before the onset of the adenopathy, she had had orogenital contact with a casual sexual partner, with exposure to orogenital secretions. Because of the possibility of LGV infection, the patient was started on IV doxycycline 200 mg, followed by a course of oral doxycycline (100 mg bid) upon hospital discharge. Within 24 hours, the patient defervesced and her cervical lymphadenitis began to resolve. She subsequently had complete resolution of her symptoms. MIF tests performed before her hospital discharge demonstrated significantly elevated Chlamydia titers (C trachomatis IgG 1:1024), compatible with acute LGV infection.
The primary lesion in LGV is a papule papule /pap·ule/ (pap´ul) a small, circumscribed, solid, elevated lesion of the skin.pap´ular
n. pl. that produces a superficial ulcer or erosion; this often heals spontaneously and is rarely present at the initial visit to the physician. (2) More commonly, patients present during the second stage of the disease with a chief complaint of prominent inguinal or femoral lymphadenopathy. (6)
Although rarely reported, prominent adenopathy in unusual locations such as the tonsil tonsil
Small mass of lymphoid tissue in the wall of the pharynx. The term usually refers to the palatine tonsils on each side of the oropharynx. They are thought to produce antibodies to help prevent respiratory and digestive tract infection but often become infected or neck, occurring after a primary oropharyngeal infection, may be the presenting symptom. (3,6,7) In one reported case, a homosexual man with a history of orogenital contact presented with prominent cervical lymphadenopathy suggesting lymphoma; subsequent serologic studies demonstrated the presence of C trachomatis, and the patient responded to tetracycline therapy. (6) In another case, a patient presented with prominent cervical lymphadenopathy and tonsillar tonsillar /ton·sil·lar/ (ton´si-lar) of or pertaining to a tonsil.
ton·sil·lar or ton·sil·lar·y
Of or relating to a tonsil, especially the palatine tonsil. hypertrophy; biopsy of the tonsil along with a positive serology suggested LGV, and the patient clinically improved on tetracycline therapy. (7)
In our patient, although no oropharyngeallesions were directly observed, the oral cavity was believed to have been the primary site of infection. This is supported by the patient's history of orogenital contact and exposure to genital secretions, the swelling of peritonsillar tissue on a cervical CT scan, and the absence of obvious genital lesions on physical examination. Fine-needle aspiration of the lymph node demonstrated granulomatous inflammation compatible with LGV; the diagnosis was further confirmed by the high titers demonstrated by chlamydial serology, as well as the patient's response to doxycycline.
LGV is rare in industrialized countries but endemic in parts of Africa, Southeast Asia, Central and South America, and the Carribean. (4,5) Even in these regions where LGV is endemic, the oral manifestations of the disease are rarely recognized and are considered relatively uncommon. (1) However, more liberal social attitudes have led to an increase in orogenital sexual activity, (3) which could lead to an increase in its incidence in the United States and other industrialized countries.
Previous reports suggest that women who are sexual contacts of men with nongonococcal urethritis rarely develop symptomatic oropharyngeal infection following fellatio A sexual act in which a male places his penis into the mouth of another person.
At Common Law, fellatio was considered a crime against nature. It was classified as a felony and punishable by imprisonment and/or death. . (8) However, in that study, investigators relied on pharyngeal chlamydial cultures to document transmission. Although these cultures are specific for C trachomatis infection, the sensitivity of this procedure for oropharyngeal chlamydial infection is unknown and may not fully reflect the true incidence of transmission.
Epidemiologic studies suggest that there is an increase in urogenital chlamydial infection, especially among adolescents and young adults. (9,10) Estimates of the incidence and prevalence of LGV in the United States are difficult to obtain, as the disease is sometimes unrecognized and often not reported. (11) Furthermore, in light of the recent LGV outbreak reported in the Netherlands, (11,12) the possibility of another such outbreak in industrialized nations should not be ignored, especially with the increase in commercial travel to and from endemic regions.
Our case suggests that oropharyngeal chlamydial infection might be more common than realized and emphasizes the need to consider the possibility of LGV in patients with unexplained cervical lymphadenopathy or tonsillitis.
Furthermore, along with initial diagnostic tests to confirm the infection (e.g., serology, lymph node biopsy Lymph Node Biopsy Definition
A lymph node biopsy is a procedure in which all or part of a lymph node is removed and examined to determine if there is cancer within the node. ), an empiric trial of doxycycline therapy given while additional confirmatory tests (e.g., NAATs) are pending may be appropriate in patients with cervical lymphadenopathy who fail to respond to standard antibiotic therapy. This is especially so if the patient has a history of orogenital contact, suggesting the possibility of exposure to C trachomatis.
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Infections that are acquired and transmitted by sexual contact. Although virtually any infection may be transmitted during intimate contact, the term sexually transmitted disease is restricted to conditions that are largely . 3rd ed. New York: McGraw-Hill; 1998:423-32.
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(10.) Satterwhite CL, Joesoef MR, Datta SD, Weinstock H. Estimates of Chlamydia trachomatis infections among men: United States. Sex Transm Dis 2008;Apr 15 [Epub ahead of print].
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(12.) Nieuwenhuis RF, Ossewaarde JM, Gotz HM, et al. Resurgence of lymphogranuloma venereum in Western Europe: An outbreak of Chlamydia trachomatis serovar L2 proctitis Proctitis Definition
Proctitis is an inflammation of the rectum.
Proctitis affects mainly adolescents and adults. It is most common in men around age 30. Proctitis is caused by several different sexually transmitted diseases. in The Netherlands among men who have sex with men Men who have sex with men (MSM) is a term used mostly in the United States to classify men who engage in sex with other men, regardless of whether they self-identify as gay, bisexual, or heterosexual. . Clin Infect Dis 2004;39(7): 996-1003.
Diana T. Albay, MD; Glenn E. Mathisen, MD
From the Department of Infectious Diseases, UCLA Medical Center, Los Angeles (Dr. Albay and Dr. Mathisen) and the Department of Infectious Diseases, Olive View Medical Center, Sylmar, Calif. (Dr. Mathisen).
Corresponding author: Glenn E. Mathisen, M D, Olive View Medical Center, Department of Infectious Diseases, 14445 Olive View Dr., 2B182, Sylmar, CA 91342. Phone: (818) 364-3205; fax: (818) 364-4573; e-mail: firstname.lastname@example.org