Skip to main content
main-content
Erschienen in: BMC Urology 1/2021

Open Access 01.12.2021 | Case report

Hard bilateral syphilitic testes with vasculitis: a case report and literature review

verfasst von: Sat Prasad Nepal, Takehiko Nakasato, Takashi Fukagai, Takeshi Shichijo, Jun Morita, Yoshiko Maeda, Kazuhiko Oshinomi, Yoshihiro Nakagami, Tsutomu Unoki, Tetsuo Noguchi, Tatsuki Inoue, Ryosuke Kato, Satoshi Amano, Moyuru Mizunuma, Masahiro Kurokawa, Yoshiki Tsunokawa, Sou Yasuda, Yoshio Ogawa

Erschienen in: BMC Urology | Ausgabe 1/2021

Abstract

Background

We report the case of a patient with syphilitic testicular gumma and vasculitis with adrenal failure due to chronic steroid use.

Case presentation

A 63-year-old male presented with hard right eye swelling and very firm bilateral testes on palpation, which he had for 2 years. Testicular tumor markers were negative; syphilis test was positive. Radiological examination suggested aortitis and bilateral testicular malignancy. The patient received ampicillin for the infection and prednisolone for vasculitis. Left orchidectomy was performed to confirm the presence of testicular tumor; histological examinations revealed granulomatous orchitis. The prednisolone doses were adjusted because of relapses and adverse effects of steroid use. Unfortunately, the patient died in the intensive care unit because of uncontrolled blood pressure and pneumonia.

Conclusions

This is a rare case of syphilis with testicular involvement and vasculitis. This report shows the importance of broadening the differential diagnoses of testicular firmness.
Hinweise

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Abkürzungen
COPD
Chronic obstructive pulmonary disease
HIV
Human immunodeficiency virus
ICU
Intensive care unit
MRI
Magnetic resonance imaging

Background

Syphilis involvement in the testis is extremely rare, and the literature has very few case reports. Testicular gummata are characterized by multiple swellings in the testis and granulomatous inflammation with a billiard ball-like hard consistency [1]. It is generally associated with patients with human immunodeficiency virus (HIV) infection. A literature search revealed this was the 24th recorded case of testicular syphilis but only the 2nd case of syphilitic vasculitis (aortitis) accompanying testicular gumma. Herein, we discuss a case of testicular gumma with vasculitis and review the current literature regarding the age at presentation, testicular characteristics, and other systemic findings of testicular syphilis.

Case presentation

In 2014, a 63-year-old patient initially presented at the medicine department with a cough and erythematous areas on the right ankle joint. The patient had a history of smoking (90 pack-years) and chronic obstructive pulmonary disease (COPD).
On examination, the bilateral testes were painless, very firm, smooth, and nonenlarged. There was bilateral decrease in breathing sounds. A painless right orbital mass—hard and immobile—was also noted, but it did not interfere with normal vision or eye movement. No other abnormal clinical signs were observed (Fig. 1).
Chest X-ray revealed bilateral pleural effusion. Thoracocentesis was normal. Testicular tumor marker levels were not increased. Rapid plasma reagent, Treponema pallidum antibody, and absorbed fluorescent treponemal antibody quantitative tests were all positive. Lumbar puncture and magnetic resonance imaging (MRI) were performed, considering the possibility of neurosyphilis. Cerebrospinal fluid findings were normal.
Computed tomography examination from the aortic arch to the descending aorta revealed an edematous and thickened wall with swollen surroundings. Aortitis was suspected (Fig. 2). Left vertebral artery stenosis and left subclavian artery dilatation were also noted (Fig. 3).
Antineutrophil cytoplasmic antibodies tests were negative. Angiographies of the abdominal cavity, kidney, and superior and inferior mesenteric arteries were performed to assess vasculitis, all of which were negative. An MRI of the ankle was performed because of erythematous lesions in the lower limbs; this revealed inflammatory findings suggesting osteomyelitis. Skin biopsy indicated dermatopanniculitis.
Ultrasound revealed uniform echogenicity across the bilateral testes (right side: 20.3 × 40.8 mm; left side: 26.6 × 42.3 mm). However, the testicular condition was inconclusive (Fig. 4). Therefore, the patient underwent another MRI.
Testes MRI showed that the bilateral testes had higher signal than the muscle on T1 imaging and lower signal than that of a normal testis on T2. Thus, malignant lymphoma of the bilateral testes was considered (Fig. 5).
Left orchidectomy was performed, and the sample was sent for histological examinations. The testis and epididymis had a uniform yellowish-white tumor. The testis was hard in consistency (Fig. 6). Microscopic findings showed that seminiferous tubules were destroyed and surrounded by lymphocytes and plasma cells. CD68-positive epididymal cells were proliferated. No foreign body giant cells or necrotic foci were observed. There were few CD20 (L26)-positive B and CD3-positive T cells; no proliferation of atypical cells was noted. No pathogens were identified on Periodic acid–Schiff, Grocott`s mehtenamine silver and Gram, or Ziehl–Neelsen staining. Granulomatous orchitis was considered (Fig. 7; We used Olympus BX51 Microscope with an objective lens of × 10, Olympus DP73 Camera, and Olympus standard CellSens standard version 1.6 as acquisition software for the microscopy.). Because the histology of left testicle was nonmalignant, the right testicle was not removed.
Our patient was diagnosed with syphilitic gumma with vasculitis. He also had a 30-year-old history of sexual activity with a sex-service worker. Ampicillin (8 g/day) for 2 weeks was started for syphilis treatment. However, prednisolone (0.8 mg/kg/day, 35 mg) was introduced and continued because of a high inflammatory response, vasculitis, and pleural effusion. However, the tests for syphilis were positive even after the antibiotic therapy.
The patient refused treatment for the eye hardness; therefore, no biopsy was performed.
The steroid dose was frequently changed as the patient had relapses (i.e., shortness of breath, pleural effusion, and pericardial fluid accumulation) upon dose reduction and experienced steroid-induced adverse effects (i.e., surgery for avascular necrosis of femoral head, uncontrolled orthostatic hypotension with lower sodium and high potassium possibly due to steroid-induced adrenal insufficiency, and steroid-induced hypogammaglobulinemia).
He complained of numbness and tingling sensation in both upper limbs after 2 years of the diagnosis. An MRI of the cervical spine revealed C3–C4 cervical spinal canal stenosis with intervertebral disc swelling and spinal cord compression as well as similar narrowing of the spinal canal in C4–C7 (Fig. 8).
In 2020, the patient was admitted to the intensive care unit (ICU) owing to a deteriorating level of consciousness and uncontrolled blood pressure. He had pneumonia due to cytomegalovirus and Pseudomonas aeruginosa infections that led to acute respiratory distress syndrome. Unfortunately, the patient died on day 18 of ICU admission.

Discussion and conclusions

This report presents a rare case of tertiary syphilis with testicular gumma and syphilitic vasculitis. It could not be controlled with antibiotics and steroids and ultimately led to adrenal dysfunction and mortality. Only a few cases of testicular involvement in syphilis have been reported. Furthermore, testicular induration as the first presentation indicating syphilis is rare.
The indurated right eye may be due to syphilis. However, the reason for the hardened eye mass remains unknown because the patient denied undergoing a biopsy. Ocular syphilis can occur in any stage of syphilis and can infect any part of the eye, with panuveitis being the most common finding. Patients generally present with loss of vision, eye pain, floaters, and photophobia [2]. Orbital involvement is rare and characterized by gumma within the orbit, extraocular muscle, or lacrimal gland and the presence of periostitis [24].
The literature review revealed that the mean age of patients with testicular syphilis at disease presentation is 42 years and five patients had HIV infection (Table 1). In total, 14 patients (14/18, 77.7%; we were unable to collect the data of six patients mentioned in the study by Archimbaud et al. [5]) presented with enlarged testis or scrotal swelling. Six patients (6/18, 33.3%) presented with a firm or hard testicle accompanied by testicular swelling. Our patient was unique in presenting with only hard bilateral testes.
Table 1
Relevant literature on testicular involvement in syphilis
Case number
Authors
Age (years)
Testicular findings
Systemic findings
1
Lees et al. 1937 [15]
27
Bilateral (first right scrotal swelling, then left scrotal swelling)
Congenital syphilis
NA
2
London et al. 1947 [17]
33
Hardening with painful swelling of testes
NA
3
Al-Egaily et al. 1977 [1]
37
Bilateral firm and enlarged testis with painless penile sores
NA
4–9
Archimbaud et al. 1984 [5]
 
Six cases
NA
10
Onishi et al. 1987 [18]
72
Right scrotal swelling with tenderness
Aortic aneurysm
Aortitis
11
Terao et al. 1993 [19]
44
Enlarged firm right testis
NA
12
Nakano et al. 1999 [20]
75
Painless left scrotal swelling
NA
13
Varma et al. 2009 [21]
39
Right testis painful and firm lump
HIV
14
Silva et al. 2010 [22]
32
Bilateral testicular swelling
NA
15
Nakano et al. 2011 [8]
47
Painful right testicular swelling
NA
16
Sekita et al. 2012 [23]
40
Left scrotal swelling
Left testis
NA
17
Teo et al. 2012 [14]
47
Right testis with firm, non-tender swelling
Non-ulcerated indurated subprepuce
Conservative treatment with doxycycline
NA
18
Liang et al. 2013 [24]
37
Left testicle and left kidney
Painless swelling
Doxycycline coz of penicillin allergy post-operation
HIV
19
Yogo et al. 2014 [25]
28
Right testis pain and swelling
Jarisch–Herxheimer reaction in the testis following Penicillin G infusion
HIV with Bilateral Retinal detachment
20
Morlacco et al. 2015 [26]
31
Right testis pain and swelling
NA
21
Chu et al. 2016 [27]
33
Right testis hardening and swelling
Non-granulomatous type
HIV
22
Tagliati et al. 2020 [13]
39
Testicular discomfort
Multiple bilateral subcentimetric hypoechoic lesions
NA
23
Agrawal et al. 2020 [28]
40
Left scrotal abscess due to epididymo-orchitis
Ulcerated enlarged left testis with indurated base
HIV
24
Our case
63
Bilateral testes induration
Aortitis
Left subclavian artery aneurysm
HIV human immunodeficiency virus, NA not applicable
The incidence of syphilis is currently increasing in developed countries. More than half of patients are males are owing to men having sex with men, and 42% of such patients were also HIV-positive [6]. The number of patients with syphilis is increasing by 1100 per year in Japan [7]; however, it is mainly because of heterosexual transmission rather than homosexual transmission [8]. It can also be caused by a direct T. pallidum (subspecies pallidum) infection and then is transmitted via blood products, either transplacentally or sexually.
Syphilis has three stages: primary (characterized by painless chancre occurring 2–6 weeks after infection), secondary (characterized by condylomata lata 1–2 months after primary syphilis), and tertiary (occurring 2–50 years after the initial infection, characterized by gummatous disease, meningovascular disease, tabes dorsalis, cardiovascular, ocular, and otic syphilis) [6].
Serologic testing is currently the standard approach for diagnosis; however, it lacks sensitivity in detecting early syphilis, congenital syphilis, neurosyphilis, tertiary syphilis, and HIV or Hepatitis C coinfection [9]. Hence, several other approaches such as immunohistochemistry (IHC), polymerase chain reaction (PCR), culture, morphological observation, and seroassay are considered for the detection of clinically undetected syphilis [10]. In a previous study, IHC had 49–92% sensitivity and excellent specificity for the diagnosis of secondary syphilis [9]. It can be used as a tool for further investigation when serological assays fail to identify the organism. However, there is a possibility of cross reaction with Borrelia burgdorferi and intestinal spirochetes [10]. PCR has a sensitivity of 89.1% in chancre specimens from patients with primary syphilis [10, 11]. In fact, the United States Centers for Disease Control and Prevention suggests that PCR is valuable for chancre samples [11]
Syphilitic gummata can mimic testicular tumors, which are usually diagnosed after surgery. Its differential diagnoses such as testicular neoplasms, mumps orchitis, tubercular epididymitis, and gonococcal epididymitis should be considered [1]. Gummata are usually multiple and regressive. It is clinically diagnosed with syphilis serology and treatment response [1]. On biopsy, obliterative endarteritis with palisading lymphocytes and plasma cells is visible. Over time, fibrous scarring causes tubular atrophy and sterility [1].
Ultrasonography shows lesions to be cystic with increased peripheral vascularity [12]. On contrast-enhanced ultrasonography, lesions show rapid wash-in and early washout [13]. Moreover, orchidectomy is generally performed for testicular syphilitic masses because of the concerns regarding missing out on the testicular tumor, thus requiring conservative management [1315].
Penicillin G is the antibiotic of choice for syphilis. However, ampicillin treatment results in a higher cerebrospinal fluid concentration in the brain than penicillin G treatment. It was used in our patient to avoid neurosyphilis, although the investigations for this were negative [8]. Although intramuscular benzathine penicillin G is the first-line antibiotic recommended by the World Health Organization, it is unavailable in Japan and carries a risk of anaphylaxis [16]. Unfortunately, the syphilis tests were not negative in our patient even after the antibiotic therapy. Thus, therapy was inadequate due to severe inflammation.
The present case highlights the importance of broadening the differential diagnoses of testicular hardening or indurations. Patients with syphilitic gummata present only with hardness; this needs to be considered for timely treatment of and complication prevention in patients with similar presentations.

Acknowledgements

The authors would like to thank Dr. Toshiko Yamochi for interpreting histology findings and Enago (www.​enago.​jp) for the English language review.

Declarations

Not applicable.
Written informed consent could not be obtained from the patient's next of kin for publication of this case report and accompanying images because his relatives could not be contacted. Therefore, patient data have been deidentified to avoid patient identification.

Competing interests

The authors declare that they have no competing interests.
Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://​creativecommons.​org/​licenses/​by/​4.​0/​. The Creative Commons Public Domain Dedication waiver (http://​creativecommons.​org/​publicdomain/​zero/​1.​0/​) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Literatur
5.
Zurück zum Zitat Archimbaud A, Bonvalet D, Levy-Klotz B, Vallet C, Civatte J, et al. Syphilitic orchiepididymitis. Apropos of a pseudotumoral case. Ann Dermatol Venereol. 1984;111:169–71. PubMed Archimbaud A, Bonvalet D, Levy-Klotz B, Vallet C, Civatte J, et al. Syphilitic orchiepididymitis. Apropos of a pseudotumoral case. Ann Dermatol Venereol. 1984;111:169–71. PubMed
18.
Zurück zum Zitat Onishi N, Wakabayashi A, Sugiyama T, Mitsubayashi S, Kurita T. A case of syphilitic orchitis with aortic aneurysm. Hinyokika Kiyo. 1987;33:1496–9. PubMed Onishi N, Wakabayashi A, Sugiyama T, Mitsubayashi S, Kurita T. A case of syphilitic orchitis with aortic aneurysm. Hinyokika Kiyo. 1987;33:1496–9. PubMed
19.
Zurück zum Zitat Terao T, Kura N, Ohashi H, Mizuo T, Kameda N. Syphilitic orchitis: report of a case. Hinyokika Kiyo. 1993;39:973–6. PubMed Terao T, Kura N, Ohashi H, Mizuo T, Kameda N. Syphilitic orchitis: report of a case. Hinyokika Kiyo. 1993;39:973–6. PubMed
20.
Zurück zum Zitat Nakano Y, Chokyu H, Inaba Y, Harada M, Gotoh A, Maeda S. Syphilitic orchitis: a case report Hinyokika kiyo. Acta Urol Japon. 1999;45:289–91. Nakano Y, Chokyu H, Inaba Y, Harada M, Gotoh A, Maeda S. Syphilitic orchitis: a case report Hinyokika kiyo. Acta Urol Japon. 1999;45:289–91.
23.
Zurück zum Zitat Sekita N, Nishikawa R, Fujimura M, Sugano I, Mikami K. Syphilitic orchitis: a case report. Hinyokika kiyo Acta Urologica Japonica. 2012;58:53–5. PubMed Sekita N, Nishikawa R, Fujimura M, Sugano I, Mikami K. Syphilitic orchitis: a case report. Hinyokika kiyo Acta Urologica Japonica. 2012;58:53–5. PubMed
Metadaten
Titel
Hard bilateral syphilitic testes with vasculitis: a case report and literature review
verfasst von
Sat Prasad Nepal
Takehiko Nakasato
Takashi Fukagai
Takeshi Shichijo
Jun Morita
Yoshiko Maeda
Kazuhiko Oshinomi
Yoshihiro Nakagami
Tsutomu Unoki
Tetsuo Noguchi
Tatsuki Inoue
Ryosuke Kato
Satoshi Amano
Moyuru Mizunuma
Masahiro Kurokawa
Yoshiki Tsunokawa
Sou Yasuda
Yoshio Ogawa
Publikationsdatum
01.12.2021
Verlag
BioMed Central
Erschienen in
BMC Urology / Ausgabe 1/2021
Elektronische ISSN: 1471-2490
DOI
https://doi.org/10.1186/s12894-021-00886-5

Weitere Artikel der Ausgabe 1/2021

BMC Urology 1/2021 Zur Ausgabe

Newsletter

Bestellen Sie unseren kostenlosen Newsletter Update Urologie und bleiben Sie gut informiert – ganz bequem per eMail.