Email updates

Keep up to date with the latest news and content from BMC Infectious Diseases and BioMed Central.

Open Access Highly Accessed Case report

Poststreptococcal syndrome mimicking conjunctival lymphoma

Iwona Rospond-Kubiak1*, Agata Brązert1, Jarosław Kocięcki1 and Jan Bręborowicz2

Author affiliations

1 Department of Ophthalmology, Poznań University of Medical Sciences, 1/2 Długa St., Poznań, 61-848, Poland

2 Department of Oncologic Pathology, Chair of Oncology, Poznań University of Medical Sciences, 1/ 2, Łąkowa St, Poznań, 61-878, Poland

For all author emails, please log on.

Citation and License

BMC Infectious Diseases 2013, 13:149  doi:10.1186/1471-2334-13-149

The electronic version of this article is the complete one and can be found online at: http://www.biomedcentral.com/1471-2334/13/149


Received:27 May 2012
Accepted:15 March 2013
Published:25 March 2013

© 2013 Rospond-Kubiak et al.; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background

Poststreptococcal syndrome (PSS) can be a consequence of nonpurulent primary infection with group A streptococci (GAS). Postreptococcal uveitis is a well recognized entity with quite a few descriptions in the literature, but so far no conjunctival involvement has been reported.

The aim of the study is to present a rare case of postreptococcal conjunctival lesions mimicking a lymphoma.

Case presentation

19-years-old Caucasian female presented with pink, nodular infiltrates in the right conjunctiva that occurred a few months after upper respiratory tract infection and tonsillectomy. Histopathological examination of collected lesion samples revealed inflammatory reaction with lymphocytes proliferation and failed to rule out a myeloma. Complementary flow-cytometry did not show monoclonal proliferation of lymphocytes B. During follow-up we observed the complete regression of conjunctival lesions after the benzyl penicillin treatment prescribed by ENT specialist due to elevated plasma ASO levels. Therefore, we suppose that those lesions must have represented a part of poststreptococcal syndrome.

Conclusions

To conclude, this is, to the best of our knowledge, the first report of conjunctival involvement in the course of PSS related to group A streptococci.

Keywords:
Postreptococcal syndrome; Anti-streptolysine 0; Conjunctival lymphoma

Background

Poststreptococcal syndrome (PSS) is a sterile inflammation thought to represent an autoimmune reaction between streptococcus-sensitized lymphocytes and host tissue because of ‘molecular mimicry’. It can be a consequence of nonpurulent primary infection with group A streptococci (GAS). The most common forms of PSS are: rheumatic fever, acute poststreptococcal glomerulonephritis and erythema nodosum.

Ocular tissues involvement in PSS has been rarely reported in literature in the form of poststreptococcal uveitis secondary to group A streptococcal infection [1-5] and keratouveitis after a pharyngitis caused by group C streptococci (GCS) [6]; but, to date, conjunctival involvement has not been reported.

Poststreptococcal uveitis can affect any part of the uvea and is almost always bilateral, nongranulomatous with the occasional formation of posterior synechiae. It appears to be a disease of young patients (96% under 40 years of age, and more than 50% younger than 15 years) [4]. There is a controversy regarding the recognition of PSS syndrome only after an elevated anti-streptococcal lysine O (ASO) titer, which may variate regarding the patient age, ethnicity and season of the year [3]. The streptozyme agglutination test (SAT) represent another diagnostic option.

The aim of the study is to present a rare case of postreptococcal conjunctival lesions mimicking a lymphoma.

Case presentation

A 19-year-old Caucasian female was referred to the Ocular Oncology Service, Department of Ophthalmology, Poznań, Poland in April 2009 with a suspicion of conjunctival lymphoma. The patient reported the appearance of painless reddish nodules on the right bulbar conjunctiva couple weeks previously. The patient didn’t have past ocular history of note. Her past medical history revealed history of purulent tonsillitis treated with systemic antibiotic therapy a year earlier. However, after this treatment the levels of antistreptolysin (ASO) were still elevated (up to 500 U/ml) and the patient had undergone tonsillectomy three months later. The working clinical diagnosis by her local ophthalmologist was of suspected lymphoma and the patient received no topical treatment. At presentation to our department, the ASO level was 482 U/ml (N: 10–200 U/ml) and other laboratory test results were within normal limits. The patient was otherwise healthy with no travelling history and reported no coincidence between the appearance of conjunctival lesions and tonsillitis.

On ocular examination, the best corrected visual acuity was 5/5 with the right eye and 5/5 with the left eye, which was healthy. On biomicroscopy there were numerous, pink and reddish nodules on the right bulbar conjunctiva nasally and temporally [Figure  1A and B]. Those lesions seemed to be tightly fixed to the sclera. There was a similar subconjunctival mass in the right lower fornix. Otherwise the anterior segment appeared normal, with no signs of uveitis or keratitis. The right fundus appeared normal. Orbital ultrasound revealed no pathology. No lymphadenopathy was noted. Because the observed lesions did not have the typical appearance of a conjunctival lymphoma and the diagnostic uncertainty, incisional biopsy was taken.

thumbnailFigure 1. (A + B) - Anterior segment of right eye (RE) – April 2009, and disappearance of conjunctival lesions after benzyl penicilin treatment in June 2009 (C + D).

The histopathological assessment of the collected specimens revealed infiltration by plasma cells and lymphocytes. The immunohistochemistry enabled recognition of B cells (CD 20 +) and T cells (CD3 +), which showed enhanced expression of Ki-67. Expression of CD 10, CD 23, CD 43 and bcl2 was also detected [Figure  2]. This might have corresponded to an inflammatory process, but also to a conjunctival infiltration in the course of multiple myeloma. As a part of further management, a bone marrow biopsy was performed. Flow cytometry revealed 25.5% lymphocytes without evidence of monoclonality, 4% monocytes, 69.5% neutrophils and 1% mieloblasts. Other systemic investigations (chest X-ray, abdomen ultrasound) revealed no pathology.

thumbnailFigure 2. Histopathologic staining of the lesion samples (A) – H + E, (B) – CD 20 (+) and (C) – Ki67

On the next follow-up visit, 2 months later, in June 2009, shrinking and pallor of the conjunctival lesions was noted. [Figure  1C and D]. The patient reported receiving treatment with benzyl penicillin (6×2.4 mln units every two weeks) 4 weeks earlier by the ENT specialist because of persistent elevation of the plasma concentration of ASO. At the next follow-up in 6 weeks time the conjunctival lesions had completely disappeared.

Discussion

In view of the clinical progress of our case, the likely diagnosis of poststreptococcal syndrome (PSS) affecting the conjunctiva that had responded to penicillin treatment was made. The differential diagnoses for that case would be anterior scleritis or episcleritis, conjunctival lymphoma, ocular infiltration in the course of multiple myeloma, reactive lymphoid hyperplasia, tuberculosis, chlamydia or herpetic infection, papilloma and superficial migratory phlyctenulosis.

The first two conditions could be ruled out since the lesions initially were painless. However, a multifocal nodular episcleritis and scleritis can sometimes appear in the course of Hodgkin’s lymphoma but the onset of a disease in the cases reported in the literature was associated with pain [7]. Similarly, the clinical findings could have been part of myeloma [8]. Although histopathologic examination revealed an infiltration by plasma cells and lymphocytes, further hematologic and systemic investigations (bone marrow biopsy and flow cytometry) ruled out multiple myeloma and other lymphoproliferative disease [9]. There was no discharge, no lymphadenopathy, no corneal involvement at first presentation and no systemic signs of infectious disease which let us to rule out tuberculosis, chlamydia or herpes infection although the collected lesion samples were not evaluated with PCR for a specific DNA.

Furthermore, we observed the diminution of all lesions after benzyl penicilin treatment due to elevated plasma concentration of ASO which seems to confirm the final diagnosis.

Conclusion

To conclude, this is, to the best of our knowledge, the first report of conjunctival involvement in the course of PSS related to group A streptococci.

Consent

Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Series Editor of this journal

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

IRK performed the incisional biopsy, was responsible for the clinical management of the patient, prepared most of the manuscript; AB collected the photograps and prepared a clinical part in the first draft of the manuscript, JK supervised the clinical management of the patient, made the literature search and set the final diagnosis, JB evaluated the histopathology. All authors revised and accepted the final version of the manuscript.

Acknowledgments

This work received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

References

  1. Hanno E, Gay D, Boyer S: Two differing presentations of chronic bilateral anterior uveitis.

    Optometry 2009, 80:70-75. PubMed Abstract | Publisher Full Text OpenURL

  2. Viel A, Kolyvras N, Catherine J: Post-streptococcal uveitis.

    J Fr Ophtalmol 2011, 34(4):256.

    e1-6

    PubMed Abstract | Publisher Full Text OpenURL

  3. Gallagher MJ, Mahiul MK, Muqit MMK, David Jones D, Gavin M: Post-streptococcal uveitis.

    Acta Ophthalmol Scand 2006, 84:424-428. PubMed Abstract | Publisher Full Text OpenURL

  4. Rehman S, Anand S, Reddy A: Poststreptococcal syndrome uveitis.

    Ophthalmol 2006, 113:701-706. Publisher Full Text OpenURL

  5. Holland GN: Recurrent anterior uveitis associated with streptococcal pharyngitis in a patient with history of postreptococcal syndrome.

    Am J Ophthalmol 1999, 127:346-347. PubMed Abstract | Publisher Full Text OpenURL

  6. Nataneli N, Aguilera ZP, Rosenbaum PS: Poststreptococcal keratouveitis associated with group C streptococcus pharyngitis.

    Clin Ophthalmol 2011, 5:1257-1259. PubMed Abstract | Publisher Full Text | PubMed Central Full Text OpenURL

  7. Thakker M, Perez V, Moulin A: Multifocal Nodular Episcleritis and Scleritis with Undiagnosed Hodgkin’s Lymphoma.

    Ophthalmol 2003, 110:1057-1060. Publisher Full Text OpenURL

  8. Chin K, Kempin S, Milman T: Ocular manifestations of multiple myeloma: three cases and a review of the literature.

    Optometry 2011, 82:224-230. PubMed Abstract | Publisher Full Text OpenURL

  9. Decaudin D, Dendale R, Lumbroso-Le Rouic L: Treatment of mucosa-associated lymphoid tissue-type ocular adnexal lymphoma.

    Anti-Cancer Drugs 19:673-680. OpenURL

Pre-publication history

The pre-publication history for this paper can be accessed here:

http://www.biomedcentral.com/1471-2334/13/149/prepub