Dermatologic Manifestations of Lymphogranuloma Venereum Clinical Presentation

Updated: May 10, 2018
  • Author: Jose A Plaza, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

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.

Primary stage

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.

Secondary stage

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.

Tertiary stage

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.

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Physical Examination

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.

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Complications

Complications usually arise from progression to the third stage of lymphogranuloma venereum (LGV). Scarring and local tissue destruction is the rule, with stricture and fistula formations and deformation of genitalia. Complete bowel obstruction from rectal stricture is possible.

Systemic spread occasionally can result in arthritis, pneumonitis, hepatitis, or, rarely, perihepatitis.

Rare systemic complications include pulmonary infection, cardiac involvement, aseptic meningitis, and ocular inflammatory disease.

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