CLINICAL RECORD

The Journal of Laryngology & Otology (2014), 128, 557–560. © JLO (1984) Limited, 2014 doi:10.1017/S0022215114001200

A rare case of a chronic syphilitic gumma in a man infected with human immunodeficiency virus S D MASEGE1, A KARSTAEDT2 1

Ear, Nose and Throat Department/ Head and Neck Surgery, and 2Department of Medicine (Division of Infectious Diseases), Chris Hani Baragwanath Hospital and University of the Witwatersrand, Soweto, Johannesburg, South Africa

Abstract Objective: This paper reports a rare case of a human immunodeficiency virus infected man with gummatous syphilis of the face. Case report: A 39-year-old man presented with an ulcer of the face which had been slowly progressive over the previous 6 years. Examination showed an ulcerative lesion of the midface involving the cheeks, and completely destroying the nose, the upper lip and part of the lower lip. The teeth and gums were exposed. The ulcer had a moist, purulent base with rolled edges. The patient had human immunodeficiency virus, with a cluster of differentiation 4 count of 641 cells per μl. The rapid plasma reagin test titre was 1:1024 and the Treponema pallidum haemagglutination assay result was positive. Biopsy showed non-necrotising granulomata with a negative Warthin–Starry silver stain. There was a dramatic response to treatment with penicillin. Conclusion: This case study is a reminder that syphilis needs to be considered in the differential diagnosis of unusual presentations involving skin and bone. Key words: Syphilis; Neurosyphilis, Gummatous; HIV; Penicillin; Pathology; Face

Introduction Late benign syphilis is rare in the antibiotic era. Even after 25 years of clinical reports of syphilis and human immunodeficiency virus (HIV) co-infection linked with possible increased progression and poorer treatment outcomes,1 there has been no report of gummatous syphilis in the HIV-infected population in 20 years,2,3 and few in the HIV-uninfected. We present a case of an HIV-infected man with chronic gummatous syphilis.

Case report A 39-year-old man presented to the ENT department at the Chris Hani Baragwanath Hospital, Soweto, South Africa, with an ulcer on the face, covered by a handkerchief. The ulcer had been slowly progressive over a period of six years. It started as a small ulcer and gradually increased in size to involve the nose, the lips and ultimately the medial aspects of both cheeks. There was an associated dry cough. The patient denied any occupational exposure to heavy metals or use of illicit intranasal drugs. There were no other lesions anywhere else on the body and no rash. The patient had recently been seen at a local clinic where a biopsy of the ulcer was performed. This showed ulceration, polymorphonuclear cell inflammatory infiltration, microabscess formation and secondary vasculitis. A Ziehl–Neelsen stain and Warthin–Starry silver stain were both negative. A diagnosis was not made.

Accepted for publication 20 November 2013

Past medical history was non-contributory and travel history was negative. The patient drank alcohol occasionally and was a smoker, with a five pack-year history. There was no history of a genital ulcer. Examination of the head and neck showed an ulcerative lesion of the midface involving the cheeks, and completely destroying the nose and the upper lip (Figures 1 and 2). The lower lip was destroyed completely on the right side; both the teeth and the gums were exposed. The palate was intact. The lesion had a moist, purulent base with rolled edges (Figure 1). The differential diagnosis included all the midline granulomatous conditions: T-cell lymphoma, Wegener’s granulomatosis, sarcoidosis, tuberculosis, deep fungal infection, Churg–Strauss syndrome and lethal midline granulomatosis disease. Skull X-rays showed the destructive nature of the lesion, with absent nasal bones (Figures 3 and 4). Blood test results for full blood count, urea and electrolytes, and liver function, were normal. The erythrocyte sedimentation rate was 114 mm per hour. The angiotensin-converting enzyme level was normal. An HIV enzyme-linked immunosorbent assay was reactive, with a cluster of differentiation 4 count of 641 cells per μl. The rapid plasma reagin test result was positive, with a titre of 1:1024. This finding was supported by a positive Treponema pallidum haemagglutination assay result.

558

FIG. 1 Frontal view of the patient with the rare benign syphilitic gumma, prior to treatment.

A biopsy of the lesion was performed and this showed non-necrotising granulomatous inflammation. The detailed report showed: ulceration, polymorph inflammatory infiltration, microabscess formation, secondary vasculitis, negative Ziehl–Neelsen results and negative results for spirochaetes on Warthin–Starry silver stain (Figure 5).

S D MASEGE, A KARSTAEDT

FIG. 3 Frontal skull X-ray, demonstrating the destructive nature of the lesion.

FIG. 4 Lateral skull X-ray (note that the nasal bones are completely destroyed by the disease).

FIG. 2 Side view of the patient with the syphilitic gumma, prior to treatment.

FIG. 5 Photomicrograph showing non-necrotising granulomata. ( H & E stain, 10×) Courtesy of Prof. M Hale , Dept. of Histopathology

CLINICAL RECORD

FIG. 6 Frontal view of the patient at day 10 of high-dose intravenous penicillin G.

FIG. 7 Side view of the patient at day 10 of high-dose intravenous penicillin G.

Tests for tuberculosis were negative. The patient refused to undergo a lumbar puncture to exclude neurosyphilis. (This possibility was considered because the patient had mood swings.) An audiogram showed normal hearing bilaterally. A diagnosis of a syphilitic gumma of late benign tertiary syphilis was made. The patient was treated with 24 million units per day (administered intravenously) of aqueous crystalline penicillin (penicillin G) for 21 days. This treatment was chosen in order to cover for possible neurosyphilis.4 Over the course of treatment, the lesions on the face improved dramatically as they dried, and were reduced in size (Figures 6 and 7). The Figure 6 photograph was taken 10 days after the initiation of penicillin. The patient was assessed by plastic surgeons for possible reconstruction of the facial defect, but the patient refused cosmetic surgery and was subsequently lost to follow up.

Discussion Syphilis is an infectious venereal disease caused by the spirochaete bacterium Treponema pallidum. If left untreated, 30 per cent of patients with latent syphilis will go on to develop tertiary syphilis.5,6 Tertiary syphilis can be

559 subdivided into late benign syphilis, cardiovascular syphilis and neurosyphilis. However, it was revealed by Nnoruka and Ezeoke that a mucocutaneous gummatous lesion of tertiary syphilis and advanced secondary syphilis can be seen simultaneously,7 indicating a rapid progression of syphilitic disease within a very short time.1,7 This supports Gonzalez and Rhodes’ statement (as cited in Nnoruka and Ezeoke7) that patients can progress from primary to tertiary syphilis in a matter of weeks if there is co-infection with HIV. In the Oslo study of the natural history of syphilis, conducted prior to the discovery of antibiotic treatment, late benign syphilis occurred in approximately 17 per cent of 1147 syphilis patients and was the commonest manifestation of tertiary syphilis.5 In that study, the condition mostly affected skin (70 per cent), followed by involvement of bone (10 per cent) and mucosa (10 per cent). Upper respiratory tract involvement was not uncommon. Gummas were seen 1 to 46 years after infection, but usually in the first 15 years, and could remit and recur later. In the antibiotic era, late benign gummatous syphilis is an unusual finding. The gumma represents a maximal inflammatory response to a few organisms. It is not infectious and it is very rare to find spirochaetes. Syphilitic inflammation is generally mild, but chronic destruction of tissue leading to fibrosis can occur. The early lesion is granulomatous and resembles tuberculosis. The gummatous lesion is usually nodular, ranging from a couple of millimetres to a few centimetres in diameter. The lesions are called gummas as the necrotic material is of a gummy consistency. On histology, these lesions show obliterative endarteritis, with central avascular necrosis; the inner lining of the artery is surrounded by a fibrous capsule that contains plasma cells and lymphocytes. The gummas appear to arise in people untreated or inadequately treated, or occur with reinfection in previously treated individuals. • Clinicians should consider syphilis in the differential diagnosis of patients with unusual lesions • Syphilis can be difficult to diagnose in a human immunodeficiency virus (HIV) infected individual • Biopsy and serology are important in making the diagnosis • Development of tertiary syphilis can be hastened by HIV infection • Sometimes primary lesions and tertiary lesions can present simultaneously Cutaneous lesions present in two different ways and may either be a superficial nodulo-ulcerative type, or a deeper, more destructive gummatous type. After weeks or months the lesions may ulcerate, leaving an atrophic scar. Bony gummas appear radiographically as areas of bone lysis with a surrounding dense sclerotic reaction, as seen in the nasal bones of the patient described here. Diagnosis is to a large extent dependent on the consideration of syphilis as part of the differential diagnosis.8,9 Syphilis is known for good reason as being ‘the great imitator’.10 It should also be kept in mind that HIV makes it more likely for syphilis to present with atypical features and an unusual pattern of disease.1,4 Serological screening tests are usually reactive, and the non-specific tests may reveal

560

S D MASEGE, A KARSTAEDT

high titres as in the current case study. Serology may be negative in solitary bone lesions. Treatment generally involves benzathine penicillin G (Bicillin® L-A), 2.4 million units, administered intramuscularly on a weekly basis, for 3 weeks. There is a good response to treatment and this can be used to add certainty to the diagnosis. The patient in this case study had a gumma that involved the nasal bones, cheeks and lips, implicating the upper respiratory tract. The gummas in the four documented patients with co-infection were on the legs in two individuals, in the groin in one and on the penis in the other.2,3 The two documented cluster of differentiation 4 counts were 642 and 426 cells per μl. Although the patient in the current study was HIVinfected, he had a preserved cluster of differentiation 4 cell count, and there was no evidence that his clinical presentation or response to treatment for late benign gummatous syphilis was influenced by his HIV status. Despite there being more than 25 years of clinical experience with coinfected patients, the interaction between syphilis and HIV remains unclear.1 More aggressive and unusual presentations occur in a few people and may represent usual variant presentations rather than being specifically HIV-related. The fact that there are so few reports of late benign syphilis related to HIV infection suggests that there is no interaction for this form of syphilis. Cerebral gummas have been reported in 11 HIV-infected people but are considered to belong to the neurosyphilis group and are also rare.11 As pointed out by other researchers, all HIV-positive patients co-infected with syphilis should be treated as if they had neurosyphilis, regardless of the stage at which they present.4

is a need to keep testing for syphilis even if initial serological test results are negative.

Conclusion

E-mail: [email protected]

This case study serves to remind clinicians of the need to consider syphilis in the differential diagnosis of unusual presentations involving skin and bone, and other organs of the body. Furthermore, in patients co-infected with HIV, there

Dr S D Masege takes responsibility for the integrity of the content of the paper Competing interests: None declared

References 1 Zetola NM, Klausner JD. Syphilis and HIV infection: an update. Clin Infect Dis 2007;44:1222–8 2 Dawson S, Evans BA, Lawrence AG. Benign tertiary syphilis and HIV infection. AIDS 1988;2:315–16 3 Hay PE, Tam FWK, Kitchen VS, Horner S, Bridger J, Weber J. Gummatous lesions in men infected with human immunodeficiency virus and syphilis. Genitourin Med 1990;66:374–9 4 Lynn WA, Lightman S. Syphilis and HIV: a dangerous combination. Lancet Infect Dis 2004;4:456–66 5 Harrison LW. The Oslo study of untreated syphilis review and commentary. Br J Vener Dis 1956;32:70–8 6 Rockwell DH, Yobs AR, Moore MB Jr. The Tuskegee study of untreated syphilis: the 30th year of observation. Arch Intern Med 1964;114:792–8 7 Nnoruka EN, Ezeoke AC. Evaluation of syphilis in patients with HIV infection in Nigeria. Trop Med Int Health 2005;10:58–64 8 Hicks CB, Benson PM, Lupton GP, Tramont EC. Seronegative secondary syphilis in a patient infected with the human immunodeficiency virus (HIV) with Kaposi sarcoma. A diagnostic dilemma. Ann Intern Med 1987;107:492–5 9 Taniguchi S, Osato K, Hamada T. The prozone phenomenon in secondary syphilis. Acta Derm Venereol 1995;75:153–4 10 Rodriguez S, Teich DL, Weinman MD, Greene JM, Keroack MA, Apstein MD. Gummatous syphilis: a reminder. J Infect Dis 1988;157:606–7 11 Fargen KM, Alvernia JE, Lin CS, Melgar M. Cerebral syphilitic gummata: a case presentation and analysis of 156 reported cases. Neurosurgery 2009;64:568–76 Address for correspondence: Dr S D Masege, Ear, Nose and Throat Department/ Head and Neck Surgery, Chris Hani Baragwanath Hospital and University of the Witwatersrand, Soweto, Johannesburg, South Africa

Copyright of Journal of Laryngology & Otology is the property of Cambridge University Press and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.

A rare case of a chronic syphilitic gumma in a man infected with human immunodeficiency virus.

This paper reports a rare case of a human immunodeficiency virus infected man with gummatous syphilis of the face...
325KB Sizes 4 Downloads 3 Views