Behcet\u27s disease is a multisystem inflammatory vascular disorder with a chronic course characterized by recurrent oral and genital ulcers, eye lesion, arthritis and skin lesions. It has a typically waxing and waning course. The cause and pathogenesis of the disease are unclear and specific treatment is not available. A 39 years old man presented with rash, ocular manifestation and left leg swelling. He was found to have deep venous thrombosis of left leg along with recurrent cerebral venous thrombosis. He was a known case of Behcet\u27s disease since 3 years and had been on anticoagulants since then

Almas, Aysha

Jafri, Lena

Nasir, Nosheen

English

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eCommons@AKUDepartment of Pathology and Laboratory Medicine Medical College, PakistanNovember 2012Multifocal venous thrombosis in behcet’s diseaseLena Jafrilena.jafri@aku.eduNosheen NasirAysha AlmasAga Khan University, aysha.almas@aku.eduFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiolPart of the Microbiology Commons, and the Pathology CommonsRecommended CitationJafri, L., Nasir, N., Almas, A. (2012). Multifocal venous thrombosis in behcet’s disease. JCPSP: Journal of the College of Physicians andSurgeons Pakistan, 22(11), 730-732.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol/475730 Journal of the College of Physicians and Surgeons Pakistan 2012, Vol. 22 (11): 730-732INTRODUCTIONBehcet's disease is a multi-system inflammatorydisorder with a chronic relapsing course. It is prevalentalong the ancient Silk Route.1 There are no patho-gnomonic laboratory tests and the diagnosis requiresrecurrent oral ulceration accompanied by two of thefollowing: genital ulceration, ocular disease, skin lesionsor a positive skin pathergy reaction.2,3 Despite theinclusive criteria set forth by the International StudyGroup, there are cases where the criteria cannot be metand a diagnosis is missed. It usually affects young adults20 – 40 years of age.4,5We report, to the best of our knowledge, the first casefrom Pakistan regarding an aggressive case of Behcet'sdisease in a 39 years old man, with ocular manifestationand venous thrombosis at multiple sites despite being onanticoagulants.CASE REPORTA 39 years old man presented to the clinic with rash onthe body for 3 days. He complained of low gradeintermittent fever and left leg swelling since 4 – 5 days.He was unable to swallow due to painful mouth ulcersand hence was admitted. He was diagnosed to haveBehcet's disease 3 years ago and had been on warfarin.In previous magnetic resonance imaging (MRI) andmagnetic resonance venography (MRV) repeated fewmonths after cerebral venous thrombosis superiorsagittal sinus was not clearly visualized in its posteriorpart, with significantly increased vascularity noted inthe near vicinity of superior sagittal sinus. Featureswere suggestive of partial re-canalization of superiorsagittal sinus following thrombosis (Figure 1). Magneticresonance arteriography (MRA) images were un-remarkable. He developed recurrent mouth ulcersprogressively increasing in frequency with > 3 episodesin a year. He was also treated with steroids during thistime. He gave a history of cigarette smoking 4 – 8/day.Patient denied photosensitivity, alopecia, weight lossand alcohol intake.On examination, he was a lethargic man of good built,afebrile with a blood pressure of 135/89 mmHg, pulserate 101 beats/minute and respiratory rate 19 breaths/minute. There were multiple papulo-pustular 1 – 2 cmlesions resembling acne on face, back, chest, groin andCASE REPORTMultifocal Venous Thrombosis in Behcet's DiseaseLena Jafri1, Nosheen Nasir2 and Aysha Almas2ABSTRACTBehcet's disease is a multisystem inflammatory vascular disorder with a chronic course characterized by recurrent oraland genital ulcers, eye lesion, arthritis and skin lesions. It has a typically waxing and waning course. The cause andpathogenesis of the disease are unclear and specific treatment is not available. A 39 years old man presented with rash,ocular manifestation and left leg swelling. He was found to have deep venous thrombosis of left leg along with recurrentcerebral venous thrombosis. He was a known case of Behcet's disease since 3 years and had been on anticoagulantssince then.Key words: Behcet’s disease.   Venous thrombosis.   Ulcer.Department of Pathology and Microbiology1 / Medicine2,The Aga Khan University Hospital, Karachi.Correspondence: Dr. Lena Jafri, D-60, Block B, NorthNazimabad, Karachi.E-mail: lena.jafri@aku.eduReceived August 15, 2011;  accepted April 18, 2012.Figure 1:  Brain magnetic resonance venography (MRV) scan of our patientwith evident cerebral venous thrombosis on coronal and sagittal views: Inboth MRV images, superior sagittal sinus is not clearly visualized withsignificantly increased vascularity seen in near vicinity of superior sagittalsinus. Imaging features are suggestive of partial re-canalization of superiorsagittal sinus following thrombosis.abmedial aspect of thighs and few were on the scrotumtoo. Oral thrush with multiple apthous ulcers upto thetonsillar pillars was seen. Left leg was swollen, red andtender. His vision was blurred and was unable to read ortell time. Fundoscopy revealed optic disc swelling in righteye and macular oedema in left eye. At the site of thecannula insertion a 2.5 mm erythematous papule wasnoticed on the forearm. Systemic examination wasunremarkable.Laboratory investigations are shown in Table I. ChestX-ray was unremarkable. On Doppler ultrasound of leftleg the left external iliac, common femoral, superficialfemoral and popliteal veins appeared non-compressible,distended and demonstrated no color filling or spectralwave formation.  Behcet's Disease was confirmed as perInternational Study Group (ISG) Criteria 1990.1 Therewas evidence of venous thrombosis at multiple sites(cerebral venous thrombosis and deep vein thrombosisof left leg veins) along with disease relapse.Enoxaparin 60 mg twice daily was overlapped withwarfarin 10 mg per day. Warfarin was gradually taperedto 5 mg and continued on 5 mg daily at discharge. Theprothrombin time and international normalized ratio(INR) monitoring showed subsequent INR to betherapeutic 2.5 – 2.7. He was managed with antifungalagents for his oral ulcers, and intravenous hydro-cortisone for the disease relapse. His pustules started tosubside and no new eruptions were noticed during hishospital stay. Azathioprine was also started on thesecond day of admission as a steroid sparing agent.Ophthalmology consult was sought and decreasedintraocular pressure in right eye and uveitis in the lefteye was affirmed. He was advised YAG laser andintraretinal avastin for his left eye. A rheumatologyconsult was taken and patient was offered infliximab tosalvage his vision. Patient refused to have treatment forhis vision and was discharged home.DISCUSSIONWe report this case of Behcet's disease with extensivedeep venous thrombosis along with thrombosis of thecortical venous sinuses, a rare manifestation of thisdisease from our part of the world. Arterial and venousinvolvement is one of the characteristics of Behcet'sdisease. Vascular involvement was seen in 8.3% casesin Iran while 16.8% and 18% cases have been reportedfrom Turkey and Saudi Arabia respectively.6-8 Studiesreport a fourteen-fold increase risk of venous thrombosisin Behcet's patients.9 Venous involvement is common inmen especially those with ocular involvement and apositive pathergy test.10It is observed that men are more likely affected byBehcet's disease. Age and gender distribution in Indiashows a male to female ratio of 1.8 and the age of onsetis 33 years. These findings are corroborated in thepresently reported case and conforms to the dataavailable from the rest of the world. However the clinicalpresentation reported from India shows involvement oforal and apthous ulceration followed by joint and skininvolvement as opposed to clinical symptoms reportedfrom China and Iran where there was no significant jointinvolvement.11To conclude, ocular and cerebrovascular thrombosis areimportant complications of Behcet's disease. These canoccur despite preemptive anticoagulation as providedin the present case. Hence, there is a need for regularfollow-up of such cases.REFERENCES1. Criteria for diagnosis of Behcet's disease. International StudyGroup for Behcet's Disease. Lancet 1990; 335:1078-80. 2. Mignogna MD, Fedele S, Lo Russo L. International diagnosticcriteria and delay of diagnosis in Behcet's disease. J Rheumatol2000; 27:2725.3. Dega H, Petit A, Gaulier A, Sigal M. Mucocutaneous criteria forthe diagnosis of Behcet's disease. J Am Acad Dermatol 1996;35:789-90.4. Wurmann P, Diaz G, Sabugo F, Soto L, Solanes F, Pino S, et al.Retrospective review of 44 Chilean patients with Behcetdisease. Rev Med Chil 2009; 137:1333-40.Table I: Laboratory investigations after admission.Laboratory parameter Result Laboratory parameter ResultHemoglobin (gm/dl) 14.9 Calcium (mg/dl) 9Mean cell volume (FL) 82.7 PT (seconds) 13.8White blood cell count (x10E9/L) 16.4 PT control (seconds) 11Neutrophil (%) 80.7% INR 1.31Lymphocytes (%) 6.9% APTT (seconds) 26%Eosinophils (%) 0.1% APTT control (seconds) 30%Monocytes (%) 12.2% Total Bilirubin (mg/dl) 0.8%Basophils (%) 0.1% Serum glutamic 66%pyruvic transaminase (IU/L)Platelets count (/µL) 307 Serum albumin (g/dl) 3.1Blood Urea Nitogen (mg/dl) 18 C-reactive protein (mg/dl) 10.6Creatinine (mg/dl) 1.0 Erythrocyte sedimentation 49rate (mm/hr)Sodium (mmol/L) 135 Lactate dehydrogenase (IU/L) 969Potassium (mmol/L) 4.7 Malarial  parasite Not seenonperipheral filmChloride (mmol/L) 104 ICT malaria NegativeBicarbonate (mmol/L) 19.3 Blood culture No growthPhosphate (mg/dl) 3.9 Urine detail report Trace proteins andketonesANA, ASMA and AMA were negativeAbbreviations: PT = Prothrombin time;  INR = International normalized ratio;  APTT = Activated partialthromboplastin time;  ANA = Antinuclear antibody;  ASMA = Anti smooth muscle antibody;AMA = Anti mitochondrial antibody.Multifocal venous thrombosis in Behcet's diseaseJournal of the College of Physicians and Surgeons Pakistan 2012, Vol. 22 (11): 730-732 731Lena Jafri, Nosheen Nasir and Aysha Almas732 Journal of the College of Physicians and Surgeons Pakistan 2012, Vol. 22 (11): 730-7325. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A,Akhlaghi M, et al. Behcet's disease: from East to West.Clin Rheumatol 2010; 29:823-33.6. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A,Akhlaghi M, et al. Behcet's disease in Iran: analysis of 6500cases. Int J Rheum Dis 2010; 13:367-73.7. Morelli S, Perrone C, Ferrante L, Sgreccia A, Priori R, Voci P, et al.Cardiacinvolvement in Behcet's disease. Cardiology 1997; 88: 513-7.8. al-Dalaan AN, al Balaa SR, el Ramahi K, al-Kawi Z, Bohlega S,Bahabri S, et al. Behcet's disease in Saudi Arabia. J Rheumatol1994; 21:658-61.9. Ames PR, Steuer A, Pap A, Denman AM. Thrombosis in Behcet'sdisease: a retrospective survey from a single UK centre.Rheumatology (Oxford) 2001; 40:652-5.10. Sarica-Kucukoglu R, Akdag-Kose A, KayabalI M, YazganogluKD, Disci R, Erzengin D, et al. Vascular involvement in Behcet'sdisease: a retrospective analysis of 2319 cases. Int J Dermatol2006; 45:919-21.11. Pande I, Uppal SS, Kailash S, Kumar A, Malaviya AN. Behcet'sdisease in India: a clinical, immunological, immunogenetic andoutcome study. Br J Rheumatol 1995; 34:825-30.

Multifocal venous thrombosis in behcet’s disease

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Multifocal venous thrombosis in behcet’s disease

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