Lymphadenitis caused by the sexually transmitted bacterium Chlamydia trachomatis.

Nicolas-Favre disease.

Formerly, in Western countries, lymphogranuloma venereum (LGV) caused by Chlamydia trachomatis serovar L1–L3 was a rare sexually transmitted disease. Recently, an outbreak of LGV and LGV proctitis among homosexual men was reported from The Netherlands and Germany (1,2,3).

Although LGV is worldwide in distribution, it is most prevalent in the tropics and subtropics (4). In the United States, infections caused by non-LGV serovars of C. trachomatis are the most common sexually transmitted diseases (5). LGV frequently was seen in heterosexuals and in members of lower socioeconomic classes (6); however more recently, it has become common in homosexual men, leading to proctocolitis with fistulae and strictures (7,8). The LGV lymphadenopathy is reported to be 10 to 20 times more prevalent in men than in women, a disproportion that is in part accounted by the fact that genital lesions go unobserved (9).

C. trachomatis is an obligatory intracellular bacterium (10). About 15 serovars of C. trachomatis have been recognized (11,12,13). Of these, the serovars A, B, and C are pathogens of endemic trachoma, D through K are associated with urethritis and/or conjunctivitis, and L1, L2, and L3 cause sexually transmitted lymphogranuloma venereum (LGV) (10,11,12,13). C. trachomatis undergoes two developmental stages. The small, infectious elementary body with stores of adenosine triphosphate enters the cell by endocytosis; the larger, replicative, and metabolically active form, the reticulate body, divides by binary fission to fill the endosome (cytoplasmic inclusion) with infectious units and glycogen (10,11,12,13).

The first manifestation appears at the site of infection after a latent period of 7 to 12 days. It consists of a painless, herpetiform lesion, erosion, or shallow ulcer of the genitals that heals without scarring and often passes unnoticed (14,15). In women, the primary lesion is often on the cervix and therefore seldom observed (9,16). The regional lymph nodes become tender and enlarged 1 week to 2 months later. The lymphadenopathy is usually unilateral but may be bilateral in up to one-third of cases (17). It is far more frequently detectable in men because the inguinal lymph nodes are usually involved. In women, the perianal or deep pelvic lymph nodes drain the area of primary infection (18,19). If both inguinal and femoral lymph nodes enlarge, the inguinal ligament produces the characteristic LGV “groove sign” (19). The nodes are initially firm, tender, and movable, then become matted and fixed to the surrounding tissues (Fig. 19.1). Rupture of the nodes leads to chronic sinus tract formation (17), but only about one-third of buboes rupture; the rest form masses or resolve (20). In women, the involvement of pelvic and iliac nodes may lead to chronic pelvic lymphangitis and lymphatic obstruction with chronic vulvar edema and rectal strictures (19). Systemic infection with fever, myalgia, and headache was reported in 60% of patients in one study (21). Extragenital infections such as phalangitis with cervical lymphadenopathy and proctitis may occur (1,3,22,23,24). Lymphogranuloma venereum may also involve the anus, fallopian tubes, synovium, heart, lungs, and central nervous system (10,11,12,13,19,22,23,24).