Lymphogranuloma venereum (inguinal lymphogranulomatosis): causes, symptoms and treatment

Medically reviewed: 4, February 2024

Inguinal lymphogranulomatosis: what is lymphogranuloma venereum?

Lymphogranuloma venereum or Inguinal lymphogranulomatosis (venereal lymphopathy, Nicola-Favre’s disease, tropical bubo) is a chronic suppurative inflammation of the lymph nodes caused by chlamydia infection.

Lymphogranuloma venereum, often abbreviated as LGV, is a sexually transmitted infection caused by certain strains of the bacterium Chlamydia trachomatis. While it may share some similarities with common chlamydial infections, LGV tends to invade deeper tissues, causing systemic manifestations that can lead to severe complications when left untreated.

In recent years, there has been a rise in reported cases globally, particularly among men who have sex with men (MSM), making it crucial to enhance awareness and understanding surrounding this lesser-known STI.
Etiology and Transmission

The causative agent of LGV belongs to the family of intracellular bacteria known as Chlamydiaceae, specifically three serovars (or subtypes): L1, L2, and L3.

These particular strains differ from those responsible for typical chlamydial genital infections due to their invasive nature, allowing them to spread beyond local mucosal sites. Unlike most STIs, which require direct sexual contact for transmission, LGV can also pass on through fomites, objects contaminated with infected bodily fluids. Common modes of transmission include vaginal, anal, or oral intercourse without proper protection or sharing sex toys with an infected individual.

Clinical Presentation of lymphogranuloma venereum

Primary Stage

Within 3-30 days after exposure, individuals might notice a small painless papule or shallow ulcer at the site of entry, typically found on external genitalia or rectal tissue. This initial lesion usually heals quickly, remaining asymptomatic in many patients and thus frequently overlooked.

Secondary Stage

Weeks to months following initial exposure, affected individuals develop regional lymphadenopathy—swollen inguinal lymph nodes commonly referred to as buboes—that may drain pus spontaneously or rupture open. Systemic symptoms such as fever, fatigue, and weight loss might accompany secondary stage manifestations.

Anorectal involvement can result in discharge, constipation, tenesmus, or even fistula formation. Pharyngitis or cervicitis may occur less frequently, albeit potentially leading to long-term sequelae like pelvic inflammatory disease (PID) and epididymoorchitis.

Tertiary Stage

Persistent or recurrent proctocolitis may cause strictures, fistulas, or chronic pelvic pain, whereas advanced disseminated LGV can involve virtually any organ system, resulting in skin granulomas, arthritis, neurological deficits, or eye conditions like conjunctivitis or uveitis.

Venereal granuloma begins with the formation and healing of erosion, after which there is purulent inflammation of the regional (usually inguinal) lymph nodes, ending with their dissection and scarring.

In the diagnosis of inguinal Hodgkin’s disease, identification of the pathogen by ELISA, PCR and RIF is used; examinations of the anorectal zone and urinary organs of the patient.

Therapy for lymphogranuloma is performed with antibiotics and immunomodulators. If it is deemed necessary, surgical intervention will be performed.

The greatest distribution of venereal granuloma was received in a tropical and subtropical climate, among the population of Africa, Latin America, Asia and Australia.

The occurrence of cases of lymphogranuloma venereum in USA is associated mainly with widely developed modern tourism. As a rule, the disease is observed in persons aged 20-40 years, leading an active sex life. In men, cases of inguinal lymphogranulomatosis are more common than in women. These are mainly sailors, military personnel, homosexuals, travelers.

Causes of lymphogranuloma venereum (Lymphogranuloma)

Previously, inguinal lymphogranulomatosis was considered a viral infection. However, studies in venereology proved that the causative agent of inguinal lymphoma granulomatosis is the bacterium Chlamydia trachomatis, which also causes the development of chlamydia.

Infection with lymphogranuloma occurs from both the sick person and the carrier of the infection. Greater epidemiological danger are women.

They have symptoms of lymphogranuloma venereum develop later, as a result of which they can transmit the infection, without having any clinical manifestations and not knowing that they are infected. Infection with inguinal lymphogranulomatosis occurs through the mucous or damaged skin of the genital organs.

The most common is the sexual transmission of infection, but contact infection through intimate items is also possible.

Symptoms of lymphogranuloma venereum ( inguinal lymphogranulomatosis)

The development of lymphogranuloma venereum occurs on average 2 weeks from the time of infection, but may occur several days or months later. In the clinical picture of the disease there are 3 periods.

The primary period of inguinal Hodgkin’s disease is characterized by the appearance of a round ulcer or erosion (primary affect) at the site of introduction of the pathogen.

Its healing takes several days and leaves no scar. Therefore, for the vast majority of patients (more than 70%), the onset of lymphogranuloma venereum passes unnoticed and does not serve as a reason for seeking medical attention.

Typically, the primary affect is located in the external genitalia, but may be localized in the anus, urethra, rectum. After oral sexual contact affect can be detected on the mucous membrane of the mouth, lips, tonsils, larynx.

The secondary period of inguinal lymphogranulomatosis occurs after 14-30 days from the first symptoms of the disease. It is associated with the spread of chlamydia through the lymphatic system leading to the development of regional lymphangitis.

In sexually transmitted infections, the inguinal and femoral lymph nodes are affected. With the localization of primary affect in the region of the mouth and larynx (in case of orally transmitted infections), the submandibular and cervical lymph nodes are affected.

The defeat of the lymph nodes in the lymphogranuloma venereum is manifested by pronounced inflammatory signs:

• an increase and tenderness of the lymph nodes,
• swelling and reddening of the skin over them.

Gradually, the nodes turn into large, lumpy tumors, soldering to the underlying tissues and losing mobility.

Over time, purulent fusion of the nodes occurs, accompanied by their softening, the formation of ulcers and fistulas, through which purulent contents come to the surface of the skin.

The process of suppuration may extend to the underlying tissues with skin lesions (pyoderma), bones (osteomyelitis), and joints (purulent arthritis).

The period of suppuration is often accompanied by a violation of the patient’s general condition:

• fever,
• chills,
• headache,
• myalgia and arthralgia.

Then there is a slow subsidence of inflammatory manifestations with a decrease in the size of the lymph nodes and scarring of the fistula.

However, the spread of the infection to nearby lymph nodes leads to a new aggravation of the inguinal lymphoma. Such a course of the disease with periods of remission and exacerbation may take several years. Then, without treatment, the inguinal lymphogranulomatosis enters the next stage.

The tertiary period of lymphogranuloma venereum is associated with severe impairment of the lymphatic circulation of the pelvic organs, leading to lymphostasis and genitorectal elephantiasis with impaired functioning of the pelvic organs.

In this period of inguinal Hodgkin’s disease, chronically occurring ulcerations of the pelvic organs (vagina, urethra, rectum) can occur, leading to the formation of fistulas of the vagina, cystic rectal fistula. Localization of ulceration in the urethra may be accompanied by urethral strictures with difficulty urinating or urinary incontinence. With the defeat of the rectum may develop ulcerative colitis, paraproctitis, intestinal obstruction.

Diagnosis of lymphogranuloma venereum

Given its protean clinical presentation, LGV warrants a high index of suspicion amongst healthcare providers treating patients presenting with compatible signs and symptoms.

Diagnostic tests generally entail nucleic acid amplification techniques (NAATs), primarily polymerase chain reaction (PCR) assays targeting specific genetic elements of LGV-causing serovars.

Serological tests evaluating antibody titers against Chlamydia trachomatis antigens lack both sensitivity and specificity for diagnosing active LGV infections. However, rising antibody levels might support prior exposure histories, informing patient management decisions.

Detection of the causative agent of the inguinal lymphogranulomatosis is carried out by bakposeva discharge urethra, vagina, rectum, punctate lymph node. Also used PCR study on chlamydia, ELISA and REEF. Biopsy of the lymph node in the area of ​​the lesion is performed for the purpose of histological examination to exclude its malignant metastasis.

To assess the condition of the pelvic organs and the exclusion of lymphadenitis of other etiology in case of suspected inguinal lymphogranuloma venereum, the venereologist needs the conclusions of related specialists:

• a gynecologist,
• a urologist,
• a proctologist,
• a lympologist.

Perhaps the following procedures are necessary:

• gynecological examination,
• colposcopy,
• urethroscopy,
• rectoromanoscopy,
• ultrasound of the pelvic organs,
• USDG of the pelvic lymphatic vessels.

Differential diagnosis of lymphogranuloma venereum is performed with inguinal lymphadenitis, genital tuberculosis, secondary syphilis, metastasis of malignant tumors to the lymph nodes in cancer of the anus, rectum, penis, testicle, vagina.

Treatment of lymphogranuloma venereum

Now that you’re familiar with the causes, transmission, and clinical presentation of lymphogranuloma venereum (LGV), let us explore the available treatment options for managing and curing this condition. It’s worth noting that a combination of accurate diagnosis, adequate antibiotic therapy, partner notification, and safe-sex counseling forms the cornerstone of effective LGV management. For maximum benefit, ensure close communication between patients and healthcare professionals throughout the entire treatment process.

Antibiotic Therapy

Being a bacterial infection, the mainstay of LGV treatment consists of specific antibiotics capable of eliminating Chlamydia trachomatis bacteria. Generally, prolonged courses of these medications eradicate established infections and prevent complications better than shorter durations. Below are several recommended antibiotics based on current scientific evidence and expert consensus.

Doxycycline

This broad-spectrum tetracycline derivative serves as the go-to drug for treating LGV due to its excellent activity against Chlamydia species. Typically, physicians prescribe oral doxycycline two times per day for 21 consecutive days. Patients must complete the full course even if symptoms disappear beforehand. Moreover, advise avoiding dairy products or calcium supplements within an hour of dosing, since they hinder absorption.

Common side effects may encompass gastrointestinal disturbances such as nausea, vomiting, dyspepsia, diarrhea, and abdominal cramping. Phototoxicity manifests as exaggerated sunburn reactions following UV light exposure, hence advocating judicious sun avoidance measures. Contraindications comprise pregnancy, breastfeeding mothers, and children younger than eight years old. Consult a doctor for alternative medication regimens under these circumstances.

Azithromycin

Another viable LGV treatment option involves macrolide antibiotics, notably azithromycin, offering comparable efficacy against Chlamydia trachomatis. Physicians often suggest taking 1 gram orally once a week for three weeks or divided dosages of 500 mg every day for one week. Notably, resistance to azithromycin exists, so discuss possible alternatives with a healthcare provider if faced with persistent or recurrent symptoms despite completion of azithromycin therapy.

Adverse events associated with azithromycin tend to be milder compared to doxycycline, mainly affecting digestive function characterized by loose stools, flatulence, and rarely, serious liver injury. Rarely, allergic responses ensue requiring immediate professional attention.

Other Antibiotics

Additional antibacterials provide valuable choices when contraindications limit either doxycycline or azithromycin utilization. Such agents include:

Levofloxacin

Fluoroquinolones exhibit potent anti-chlamydial action, although experts reserve them mostly for refractory instances where compliance concerns arise. Adults normally consume 500 milligrams of levofloxacin once daily for 21 straight days. Admonish caution due to infrequent yet significant risks of peripheral neuropathy, tendinitis, QT interval prolongation, Achilles tendon damage, and phototoxicity.

Erythromycin

Macrolides serve well in pediatric settings because of safety advantages over doxycycline. Although experiencing slightly reduced effectiveness versus newer counterparts, it remains a reliable fallback choice. Providing 500 mg four times daily for a span of twenty-one days offers satisfactory cure rates accompanied by minimal side effects, namely gastrointestinal distress and rare allergic reactions.

Partner Management

Patient contacts merit simultaneous evaluation and treatment irrespective of symptom presence. Timeliness diminishes prospective transmissions and circumvents consequential repercussions. Thorough discussion concerning safe-sex principles reinforces relapse avoidance and prevents future outbreaks.

Treatment is combined with fortifying and immunomodulatory therapy. In advanced cases of inguinal lymphogranulomatosis, with the formation of fistulas, strictures of the urethra or the development of intestinal obstruction, surgical intervention is required.

Prevention Strategies

As with all STIs, safe sex practices represent the cornerstone of primary prevention efforts. Consistently using barrier methods (condoms, dental dams) significantly reduces transmission risk. Regular screening for STIs allows early detection and prompt intervention, minimizing subsequent health consequences associated with delayed diagnosis or undertreatment.

Furthermore, public education campaigns raising awareness regarding LGV’s existence, diverse presentations, and susceptible populations encourage appropriate referral patterns and diagnostic workup.

In summary, recognizing Lymphogranuloma Venereum as a distinct entity requires clinicians to maintain vigilance amidst increasing prevalence rates worldwide. Its multifarious manifestations necessitate thorough evaluation, utilizing sensitive molecular diagnostics coupled with targeted therapeutic approaches addressing underlying etiologies. By fostering informed decision-making around protective behaviors, we facilitate timely identification, management, and ultimately, reduction of downstream complications linked to LGV.