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Sir,We read with great interest the case report by Kakar et al 1 describing a young man with repeated thromboses. We would, however, like to draw to your attention a serious omission in this article. The clinical description of oral ulceration, erythema nodosum and thrombotic episodes (in particular superior sagittal sinus thrombosis) is highly suggestive of Behcet's disease. This diagnosis is frequently missed in cases of recurrent thrombosis and should be highlighted here, as therapeutic intervention by, for example, cyclosporin A or cyclophosphamide, may have prevented further thromboses and potential mortality.
In Behcet's disease thrombosis, usually venous and occasionally arterial, occurs in 10–25% of patients, usually presenting as thrombophlebitis in thighs or calves. In a minority of cases, large veins are involved, including superior and inferior vena cava, superior sagittal sinus and hepatic veins. Amongst those with significant vascular disease, 25% are venous, 7% arterial and 68% combined venous and arterial disease.2
Venous thrombosis in Behcet's disease is associated with a non-specific vasculitis of large veins which on histopathological examination reveals perivascular lymphocytic infiltration.3 Finally, Kakar et al 1 mention an association with familial thrombosis, with Factor 5 Leiden accounting for 20% of cases. However, they fail to mention that up to 37.5% of patients with Behcet's disease are positive for Factor 5 Leiden, compared with 5% of control patients with rheumatoid arthritis.4 However, we believe that Behcet's disease remains a strong possibility for the underlying diagnosis in this patient and should be actively considered in similar clinical scenarios with the institution of immunosuppressive therapy in parallel with anticoagulants.
This letter was shown to the authors who responded as follows:
Sir,We appreciate the thought-provoking diagnosis of Huchinson et al for our case. Behcet's disease was considered in the differential diagnosis in our patient but was ruled out as the patient did not fulfil all the required criteria for the same. The diagnosis of Behcet's disease should be based on the International Study Group (ISG) diagnostic criteria,1-1 and not clinical judgement alone. ISG criteria include recurrent oral ulcers and two of the following: recurrent genital ulcers, eye involvement, skin lesions and positive pathergy test. The sensitivity of this set of criteria for the diagnosis of Behcet's disease is 95% and the specificity is 98%. Eye involvement in the form of uveitis (anterior or posterior) or cells in vitreous fluid on slit lamp examination or retinal vasculitis was not present in our patient. Our patient also did not have genital ulcers or positive pathergy test. Thrombosis, although an important manifestation of this condition, is not a diagnostic criteria.