Lymphogranuloma Venereum with Bilateral Bubo: A Case Report
- 1. Department of Dermatology, University of São Paulo, Faculty of Medicine, Brazil
Abstract
Lymphogranuloma Venereum (LGV) is a sexually transmitted infection caused by the invasive intracellular bacteria Chlamydia trachomatis serovars L1–L3. LGV is distributed worldwide and most prevalent in the tropics and subtropics, but also causes outbreaks in developed countries within risk groups. LGV causes a systemic disease and most commonly begins with transient, painless genital papules or ulcers, and later on leads to excessive regional lymphadenopathy. In men having sex with men (MSM), this presentation is uncommon, and colitis or proctocolitis is the predominant sign of infection. Diagnosis is based on a combination of clinical presentation, epidemiologic findings, and Nucleic Acid Amplification Techniques (NAAT). Treatment of choice is doxycycline 100 mg or 200 mg once daily for 21 days.
We report a case of lymphogranuloma venereum in an HIV-positive patient who had bilateral inguinal involvement, a very uncommon occurrence, associated with cutaneous lesions of molluscum contagiosum. He underwent therapy with doxycycline 100 mg/day for 30 days, showing a significant reduction in the inguinal buboes, but died as a result of cerebral cryptococcosis.
Keywords
• Lymphogranuloma Venereum, Diagnosis; Treatment; Chlamydia trachomatis; Coinfection
CITATION
Belda W, Jr, de Carvalho CHC (2024) Lymphogranuloma Venereum with Bilateral Bubo: A Case Report. Clin Res Infect Dis 8(1): 1066.
ABBREVIATIONS
LGV: Lymphogranuloma venereum; US: United States; UK: United Kingdom
INTRODUCTION
Lymphogranuloma venereum is an infectious, inflammatory and invasive disease of the urogenital tract, caused by Chlamydia trachomatis. Currently, lymphogranuloma has become an important cause of anogenital disease among men who have sex with other men [1]. Lymphogranuloma venereum is a sexually transmitted disease caused by L1, L2, and L3 serovars of Chlamydia trachomatis, in contrast to the A-C serotypes of this agent responsible for eye infections such as trachoma . Infections with chlamydia serovars D to K are typically asymptomatic, When present, symptoms tend to be consistent with localized mucosal inflammation (eg, urethritis, dysuria, proctitis, cervicitis, atypical vaginal discharge) [2]. Infection with the L1, L2 and L3 serotypes causes considerable disturbance in the regional lymph nodes, creating a characteristic clinical picture of painful swelling in the lymph nodes, particularly inguinal lymph nodes. Chlamydia trachomatis mainly infects the lymphatic vessels and is transmitted by vaginal, anal or oral sexual contact during unprotected sex.
Estiomene is a late complication resulting from primary infection of the lymphatics of the scrotum, penis or vulva, which causes progressive lymphangitis and chronic edema, with sclerosis and fibrosis of the subcutaneous tissue, resulting in hardening of the skin and increased volume of the affected region, often culminating in ulceration, with the male genitalia being less affected by estiomene [3]. The disease in primary stage may go undetected when only a painless papule, pustule, or ulceration appears. The clinical syndrome of lymphogranuloma was initially described almost 100 years ago, before the demonstration that Chlamydia species were its etiological agent [4]. In 1930 the Chlamydia that causes lymphogranuloma was isolated from buboes, inoculated and cultivated in monkey brains, and in 1935 it was incubated for the first time in embrocated eggs [5]. Rectal involvement by the disease was well established as one of the manifestations in 1936, when Kornblith presented his clinical and pathological findings in his 60 patients [6].
Its etiological agent is an obligatory intracellular gram- negative bacterium, which during its development cycle alternates between two forms: the infectious elementary body and the non-infectious replicative form. Chlamydia trachomatis has 15 serotypes. Serotypes L1-L3 cause lymphogranuloma, and serotype L2 can be further separated into L2, L2a and L2b, according to small differences found in their amino acid components. Serotypes A, B, Ba and C are responsible for trachoma and serotypes D to K for anogenital infections [7]. LGV symptoms are classically divided into 3 stages: local infection (primary stage), regional dissemination (secondary stage), and progressive tissue damage (tertiary stage) [8]. The disease in primary stage may go undetected when only a painless papule, pustule, or ulceration appears. The secondary stage the regional lymph nodes and sometimes the anorectum; and late sequelae, affecting the genitals and/or rectum, comprise the tertiary stage.
Women often have primary involvement of upper vagina, cervix, or posterior urethra; however, if they are receptive to anal sex they may have primary involvement of the rectum. These regions drain to the deep iliac or perirectal nodes, and cause intra-abdominal or retroperitoneal lymphadenopathy, that may lead to symptoms of lower abdominal pain or low-back pain [7-10]. Diagnostic confirmation and identification of Chlamydia trachomatis is usually performed through NAAT test using PCR followed by the LGV biovar-specific DNA NAAT test [11-14]. The differential diagnoses for the primary lesions depend on their presentation, and include herpes, syphilis, genital warts, pearly penile papules, molluscum, other bacterial and fungal infections, The differential diagnosis for localized inguinal or pelvic lymphadenopathy includes herpes, syphilis, gonorrhea, lower- limb infections, lymphoma, and pelvic malignancy [15].
CASE PRESENTATION
A 32-year-old sex worker reported that approximately 4 months ago he noticed the appearance of small, shiny, asymptomatic, circular lesions on his penis and scrotum. After approximately 1 month, an exulcerated lesion appeared on the tip of the penis, which presented a discreet serous secretion, also asymptomatic, and after 2 weeks he began to present an increase in volume, in the bilateral inguinal region, painful, which evolved to spontaneous drainage of serous-purulent material. He denies the use of topical or systemic medications, reporting that he only cleaned the lesions with soap and water. He reported working in a nightclub for years and having sex with other men, most of the time without using condoms. He presented to our service presenting round, small, shiny, asymptomatic lesions, affecting the base of the penis and scrotum, clinically compatible with molluscum contagiosum, later confirmed by histological examination of the lesions.
Along with these lesions, there was an exulcerated rounded lesion, with a slightly infiltrated base, with discreet serous secretion, on the posterior portion of the penis, accompanied by bilateral, erythematous, painful inflammatory adenopathy, with more intense serous purulent secretion on the right side. (Figure 1) He denies fever, but reports a continuous feeling of general malaise, self-medicating with common painkillers.
Figure 1: Molluscum contagiosum lesions in the scrotum, ulcerated lesion in the distal portion of the penis and bilateral secretory bubo in an HIV-positive patient.
Denies changes in intestinal functions the clinical hypothesis of lymphogranuloma venereum was made in view of the patient’s condition, and the probable association with immunodeficiency virus infection due to the fact that the patient had molluscum contagiosum lesions at the same time and the patient’s risky sexual behavior. Serological tests for syphilis and viral hepatitis were negative. HIV serology was positive, showing 80 cells/mm3 in the CD4 count. A C. trachomatis NAAT test was performed using PCR followed by the LGV biovar-specific DNA NAAT test from the same sample used in first step test.
For the detection of LGV serovars of C. trachomatis a swab was taken from the penile ulcer, and an aspirate from the inguinal bubo to perform nucleic acid amplification tests (NAATs), as well as an oral swab collection and urine test for PCR testing. As the clinical presentation was very suggestive, a complement fixation test was performed, which showed a titer of 1/256. The Chlamydia trachomatis test was positive in the material collected from the genital ulcer and the bulb, and negative in the pharyngeal swab. The PCR performed on urine was negative once the diagnosis of lymphogranuloma venereum was confirmed, the patient was treated with doxycycline 100 mg/day for 30 days.
The molluscum contagiosum lesions were removed, with the diagnosis confirmed by histopathological examination. The patient was referred to the Infectious Diseases Division for follow- up and treatment of the immunodeficiency virus. After 30 days of treatment, he was clinically reassessed and, on this occasion, showed significant regression of the bilateral adenopathy, which no longer had an inflammatory process or secretion. He also reported slight local discomfort on palpation, but there was a significant involution of the case. He also reported having started treatment with TARV at the Infectious Diseases Department, informing, however, that he was taking medications irregularly due to his work hours not being regular. Patient did not return for follow-up after 60 days of starting treatment. In contact with the Infectious Diseases Division, they informed us that the patient died as a result of cerebral cryptococcosis resulting from immunodeficiency.
DISCUSSION
Lymphogranuloma venereum is a condition caused by invasive serovars of Chlamydia trachomatis (L1, L2, or L3). Classically, is characterized by the development of transient genital ulcer(s) or papule(s), followed by the appearance of tender inguinal lymphadenopathy, most commonly unilateral, with a characteristic “groove sign” formed by swollen, matted lymph nodes developing along the course of the inguinal ligament. Untreated, the infection may lead to long-term complications such as deep tissue abscess formation, strictures, fissures, and chronic pain [16]. A common condition in men who have sex with men and with promiscuous sexual behavior, often associated with infection by the immunodeficiency virus. Differential diagnoses should be made, depending on the stage of the disease, including herpes, syphilis, genital warts, pearly penile papules, molluscum, other bacterial and fungal infections in the initial stage, and herpes, syphilis, gonorrhea, lower-limb infections, lymphoma, and pelvic malignancy in the secondary stage [15-17].
For treatment, the Canadian, US, UK, and European guidelines recommend 100 mg of oral doxycycline twice daily for 21 days as a first line of treatment [18-22]. Other drugs are also suggested for its treatment such as erythromycin 500 mg four times a day orally for 21 days. These drugs and respective therapeutic regimens are indicated for patients in these respective regions. Azithromycin in single- or multiple-dose regimens has also been proposed, but evidence is lacking to currently recommend this drug [23-25]. Worldwide, LGV is thought to account for 2–10% of genito-ulcerative disease in areas such as India and Africa. In Western regions LGV is endemic among MSM, mainly those co-infected with HIV. Heterosexual transmission of the LGV associated L2b strain has been described [26,27].
In the present case, we report an unusual condition of lymphogranuloma venereum with bilateral involvement of the nodes in the inguinal region, since the most common condition is unilateral involvement, even in the presence of the initial active lesion in the posterior portion of the penis,, and that there was no oral or rectal involvement, as well as the presence of lesions of molluscum contagiosum in a patient with acquired immunodeficiency virus. The patient responded adequately to treatment with doxycycline, but died from cerebral cryptococcosis resulting from immunodeficiency.
CONCLUSION
Despite the extraordinary advances in HIV diagnosis and treatment that have occurred in recent years, with improvements in the quality of life and prognosis of this disease, and the various campaigns to provide guidance to the general population, this epidemic has not been fully controlled and persists among us. Intercurrences and complications arising from it continue to occur, as in the case of lymphogranuloma venereum and fatal evolution due to cerebral cryptococcosis. In this way, health authorities and doctors in general must remain attentive and vigilant, not allowing it to fall into oblivion, maintaining clarification and prevention campaigns, particularly for populations with risky sexual behavior.
ACKNOWLEDGEMENTS
Walter Belda Junior: Case monitoring, literature review, preparation of the manuscript and final review of the text. No grant are involved.
Caroline Heleno Chagas de Carvalho: Case monitoring, literature review, preparation of the manuscript and final review of the text. No grant are involved.
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