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Figure 1.Evanescent rashes on the upper limbs (A and B)

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Presentation on theme: "Figure 1.Evanescent rashes on the upper limbs (A and B)"— Presentation transcript:

1 Figure 1.Evanescent rashes on the upper limbs (A and B)
A rare ocular manifestation of Adult Onset Still’s Disease: Purtscher’s-like retinopathy Cihan Buyukavsar1, Ergenekon Karagoz2, Murat Sonmez1, Eyup Duzgun1, Abdullah Kaya1, Taner Kar1, Yildiray Yildirim1 1GATA Haydarpasa Training Hospital, Department of Ophthalmology, Istanbul, Turkey 2GATA Haydarpasa Training Hospital, Department of Infectious Diseases and Clinical Microbiology, Istanbul, Turkey   CASE PRESENTATION A 29 year-old-man was admitted with a 3 week history of high fever,sore throat, rashes on the trunk and proximal extremities,reported multi-joint swelling and pain lasting for 1 week.Before being admitted to our hospital, the patient had been administered oral antibiotics (cefixime 400 mg once a day perorally)but his complaints did not dissappeared after the antibiotic treatment.On the contrary, his joint pain and swelling was obvious, and had increased on his wrists and knees.Additionally, during admission, the patient developed blurred vision and was referred to Opthalmology department for further clinical evaluation. His previous medical and family history was unremarkable. Biomicroscopic examination showed 1+ cells in the anterior chamber and anterior vitreous bilaterally. Dilated fundus examination findings revealed; macular edema and surface wrinkling with cottonwool spots at right eye and less severe,smaller cottonwool spots at left eye. Optic coherens tomography was performed and showed retinal thickness in cross-sections of the extramacular lesions and retinal thickness and subretinal fluid consistent with macular edema in cross-sections of the macular lesion in the right eye. Laboratory evaluation performed during admission showed; leucocytosis:14,200/uL( ),Hb: 11.4 g/dL ( g/dL), Hct: 35.1% (42-52); thrombocyte: 150/mm3( ),Prothrombin time (PT): 12.9sec (10-14 sec), International Normalized Ratio (INR):0.99(<2) a high erythrocyte sedimentation rate:34 mm/hour(0-15), high C- reactive protein level:177 mg/L(<0.8mg/L), elevated liver enzymes; Aspartat aminotransferase (AST):151U/L(5-34), alanin aminotransferase (ALT):152 U/L(0-55), and extremely elevated serum ferritin levels:2230 ng/ml( ).The serological tests for salmonella, toxoplasma, brucella, syphilis, rubella, influenza, HSV, EBV and CMV were found to be negative. Antinuclear antigen (ANA), ASMA, rheumatic factor (RF),Antistreptolysin O (ASO),and anti-neutrophil cytoplasmic antibody (ANCA) were also negative.Patient was finally diagnosed as AOSD by Internal Medicine and Infectious diseases departments according to Yamaguchi criteria. In the light of this diagnosis, ophthalmologic evaluation revealed that ocular findings were compatible with Purscher’s-like retinopathy.Deflazacort was initiated 90mg/day perorally with topical prednisolon six times a day and tapered off gradually in 8 weeks. As soon as initiated medication, the patient responded to the treatment so dramatically and at the beginning of the fourth week best corrected visual activity was full for both eyes and bilaterally anterior chambers were quiet with the regression in fundus and optic coherens tomography. A B Figure 2. Fundus photographs shows macular edema and surface wrinkling,2 active retinitis lesions on inferior and superior temporal retina also 1 active retinitis lesion on inferior nasal retina at right eye(A) and totally 2 active retinitis lesions on inferior and superior temporal retina at left eye(B). Figure 1.Evanescent rashes on the upper limbs . CONCLUSION In conclusion, AOSD, which is characterized by nonspecific clinical findings such as high fever, arthritis and rash, should be considered in the differential diagnosis of the cases presented with fever of unknown origin and blurred vision. Multidisciplinary approaches could prevent severe complications and provide us early diagnosis and help us to initiate treatment on time.


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