Bilateral Hemophthalmus — the First Symptom of Behcet’s Disease (Clinical Case)

Behcet’s disease (BD) — a systemic chronic idiopathic inflammatory recurrent disease of unknown etiology. The development of the disease involve a combination of various factors, among them the most important is genetic and infectious. Eye lesion is one of the most frequent and severe symptoms of Behcet’s disease. According to the literature, eye lesion appears in 54–90% cases. There are several types of Behcet’s disease: the front, posterior and panuveitis, retinal vasculitis, occlusion of the branches of the central retinal vein, episcleritis, scleritis, keratitis, anterior ischemic neuropathy, paralysis of the extraocular muscles, swelling of the macula. In 95% cases the process is bilateral, but the clinical signs are often asymmetrical. Ocular pathology usually develops after 2–3 years the onset of symptoms BD. We havn’t met in the literature any case of Behcet’s disease, which has begun with hemophthalmia. Taking all these points together it seems to us interesting to present a clinical case of Behcet’s disease which has debuted with a bilateral hemophthalmus. This article describes a clinical case of Behcet’s disease in a patient of 38 years old. The interest of this observation is caused by feature of the appearance of clinical symptoms. Bilateral hemophthalmus has originally appeared, twice (in 2013 and 2014) and has been treated. During the next hospitalization the treatment has produced the positive effect was less  pronounced. After that, patient has undergone bilateral vitrectomy (2014). In April 2015 there were recurrent mouth ulcers, and 3 months later groin ulcers has appeared, pain in the joints. The ophthalmologist examination has shown commenmorative signs of left uveitis. The patient was counseled at the Institute of Rheumatology, where were determined the diagnosis of Behcet’s disease.This paper shows the clinical data and the results of additional tests (OCT, fluorescein angiography, ultrasonography), the results of blood biochemical examination and immune status.

Behcet’s disease, hemophthalmus, fluorescein angiography, vitrectomy, ulcer of the oral mucosa and groin area

1. Ermakova NA [The clinical unit, diagnostics, pathogenesis and treatment of ocular symptoms of Behcet’s disease]. Klinika, diagnostika, jetiopatogenez i lechenie glaznyh projavlenij bolezni Behcheta. [Clinical Ophthalmology]. Klinicheskaja oftal’mologija. 2002;1:12-16. (in Russ.).

2. Manischenkova YA, Kolomiets VI Nekrasov NB, Vertiy OA, VV [KornienkoFeatures of diagnostics of Behcet’s disease]. Osobennosti diagnostiki bolezni Behcheta. [Ukrainian rheumatological journal]. Ukrainskiy revmatologicheskiy zhurnal. 2013;1:96-99. (in Russ.).

3. Alekberova ZS [Behcet’s disease (lecture]). Bolezn’ Behcheta (lektsiya). [Scientific and practical rheumatology]. Nauchno-prakticheskaya revmatologiya. 2013;1:52–58. (in Russ.).

4. Kitaichi N., Miyazaki A., Iwata D., Ohno S., Stanford M.R., Chams H. Ocular features of Behcet’s disease: an international collaborative study. Br. J. Ophthalmol. 2007 Dec;91(12):1579-82.

5. Ulusan Z., Karadag A.S., Tasar M., Kalender M., Darcin O.T. Behcet’s disease and cardiovascular involvement: our experience of asymptomatic Behcet’s patients. Cardiovasc J. Afr. 2014 Mar-Apr;25(2):63-6.

6. Cho S.B.,Cho S., Bang D. New insights in the clinical understanding of Behçet’s disease. Yonsei Med J. 2012 Jan;53(1):35-42.

7. Shamov IA Abdulpatahov DD Gitinova ZH, Abdulpatahova EE, Dadaeva BN SS [Aligadjiev Behcet’s disease in Dagestan]. Scientific and practical rheumatology Bolezn’ Behcheta v Dagestane. [Scientific and practical rheumatology]. Nauchnoprakticheskaya revmatologiya. 2004;4:39-41. (in Russ.).

8. Al-Mujaini A., Wali U.K.Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease. Indian J. Ophthalmol. 2011 May-Jun;59(3):240-1.

9. Skef W., Hamilton M. J., Arayssi T. Gastrointestinal Behçet’s disease: a review. World J Gastroenterol. 2015 Apr 7;21(13):3801-12.

10. Yazisiz V.Similarities and differences between Behçet’s disease and Crohn’s disease. World J Gastrointest Pathophysiol. 2014 Aug 15;5(3):228-38

11. Duzgun N., Kucuksahin O., Atasoy K.S., Togay Isikay C., Gerede D.M., Erden A., Sahap S.K., Ibis M.A., Ates A.Behçet’s Disease and Intracardiac Thrombosis: A Report of Three Cases. Case Rep Rheumatol. 2013;2013:637015. doi: 10.1155/2013/637015. Epub 2013 Jul 2.

12. Alekberova ZS [Behcet’s disease of childhood]. Bolezn’ Behcheta u detej. [Current Pediatrics]. Voprosy sovremennoy pediatrii. 2009;6:64-70. (in Russ.).

13. Paovic J, Paovic P, Sredovic V. Behcet’s disease: systemic and ocular manifestation//Bio Mesd Research International Volume 2013 (2013), Article ID 247345, 7 pageshttp://dx.doi.org/10.1155/2013/247345

14. Zakka F.R., Chang P.Y., Giuliari G.P., Foster C.S. Current trends in the management of ocular symptoms in Adamantiades-Behçet’s disease. Clin. Ophthalmol. 2009;3:567-79.

15. Owlia M.B., Mehrpoor G.Behcet’s Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment. ISRN Pharmacol.2012;2012:760484. doi: 10.5402/2012/760484. Epub 2012 Mar 8.

16. Park U.C., Kim T.W., Yu H.G. Immunopathogenesis of ocular Behçet’s disease // Journal of Immunology Research Volume 2014 (2014), Article ID 653539, 13 pageshttp://dx.doi.org/10.1155/2014/653539

17. Hisamatsu T., Naganuma T., Matsuoka K., Kanai T.Diagnosis and management of intestinal Behçet’s disease. Clin J Gastroenterol. 2014 Jun;7(3):205-12.

18. Ucar-Comlekoglu D., Fox A., Sen H.N. Gender Differences in Behçet’s Disease Associated Uveitis. Journal of Ophthalmology Volume 2014 (2014), Article ID 820710, 8 pages http://dx.doi.org/10.1155/2014/820710

19. Saadoun D., Wechsler B.Behçet’s disease. Orphanet Journal of Rare Diseases 2012, The electronic version of this article is the complete one and can be found online at: http://www. ojrd.com/content/7/1/20

20. Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet’s Disease. Pathology Research International Volume 2012 (2012), Article ID 690390, 9 pages http://dx.doi.org/10.1155/2012/

21. Mishima S., Masuda K., Izawa Y., Mochizuki M., Namba K. The eighth Frederick H. Verhoeff Lecture. presented by saiichi mishima, MD Behçet’s disease in Japan: ophthalmologic aspects. Trans Am Ophthalmol Soc. 1979;77:225-79.

22. Atmaca L.S., Sonmez P.A. Fluorescein and indocyanine green angiography findings in Behçet’s disease. Br. J. Ophthalmol. 2003 Dec;87(12):1466-8.

23. Kump L.I., Moeller K L., Reed G.F., Kurup S.K., Nusenblatt R.B., Levy-Clark G.A. Behçet’s disease: comparing 3 decades of treatment response at the National Eye Institute. Can. J. Ophthalmol. 2008 Aug;43(4):468-72.

24. Kahloun R., Ben Yahia S., Mbarek S., Attia S., Zaouali S., Khairallah M.Macular involvement in patients with Behçet’s uveitis . J. Ophthalmic Inflamm Infect. 2012; Sep; 2(3):121–124.

25. Talat L, Lightman S, Tomkins-Netzer O. Ischemic Retinal Vasculitis and its Management. Journal of OphthalmologyVolume 2014 (2014), Article ID 197675, 13 pages http://dx.doi.org/10.1155/2014/197675

26. Kwon S.J., Park D.H., Shin J.P.Frosted Branch Angiitis as Ocular Manifestation of Behçet’s Disease: Unusual Case Report and Literature Review. Korean J Ophthalmol. 2013 Dec;27(6): 466–469.

27. Abu-Ameerh M.A., Mohammed S.F., Mohammed M.T.,Ababneh O.H., AL-Bdour M.D. Ocular manifestations of Behçet’s disease in Jordanian patients. Saudi J. Ophthalmol. 2013 Oct;27(4):247-51.