Superficial thrombophlebitis with prominent veins 

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... leukocyte count and thrombocyte count were 11.8 g/dl, 12,400/mm 3 and 3,50,000/mm 3 , respectively on presentation. Erythrocyte sedimentation rate was 47 mm/hour. Tests for antinuclear antibodies (ANA), rheumatoid factor (RF), anti dsDNA and anti-neutrophil cytoplasmic antibodies (ANCA) were negative. Patient was started on oral Diclofenac, Paracetamol and intravenous Ceftriaxone. On the second day of his inpatient stay, approximately 24 hours after a needle prick for infusion of normal saline, he developed massive swelling and severe pain of both lower and upper extremities along with pain and swelling at the prick site. Clinical examination revealed severe tenderness of the extremities with prominent superficial veins ( Figure 3 ) and palpable tender nodules. All hand and foot joints were swollen and tender with stiffness. A color doppler examination done at this time showed incompetent and dilated saphenous, and femoral veins within the entire course with extensive thrombophlebitis of the superficial veins. Deep veins and arteries were normal. Thrombophilia testing was negative which included antithrombin III, protein C, protein S, homocysteine, activated protein C resistance test and antiphospholipid antibodies. Biopsy from a single nodule on the shin showed infiltration by neutrophils in the vessel walls. A pathergy test was done keeping in mind, the possibility of Behçet’s disease which turned out to be positive. A slit lamp examination showed no uveitis. With this clinical picture, a diagnosis of Behçet’s disease was made and patient was started on intravenous Dexamethasone 8 mg eight hourly and continued for three days. Triamcinolone buccal paste was started for painful oral ulcers. The patient’s high fever present throughout the first two days of his hospitalization subsided after starting steroids. Oral Prednisone (1 mg/kg/day) was started on fourth day. Limb edema, thrombophlebitis and pain also responded to this treatment. Rash gradually faded over one week. The patient was discharged on oral Prednisone. Behçet’s disease is a rare form of vasculitis of obscure etiology characterized by a triad of recurrent oral aphthous ulcers, genital ulcers and uveitis. As there is no specific test for the definite diagnosis of BD, the diagnosis of this disease depends on the clinical criteria. 1 It is now recognized as a multi-system disorder manifesting with attacks of mucocutaneous lesions, arthritis, venous thrombosis, arterial aneurysms, intestinal ulcers, pulmonary lesions and central nervous system lesions. 2 Characteristic manifestations of Behçet’s disease are recurrent, which may last a few days to several weeks. Mucocutaneous findings, including oral and genital ulcers, erythema nodosum-like vasculitic nodular lesions, acneiform lesions, papulopustular lesions and superficial thrombophlebitis, are the most frequently observed features of Behçet’s disease. The pathogenesis of Behçet’s disease is unknown. An enhanced and dysregulated immune response has been suggested as the underlying pathology, and this can be triggered by environmental agents, mainly microbes, in genetically susceptible individuals. 3 Behçet’s disease has been strongly associated with HLA-B51, and this association has been confirmed in different ethnic groups . 4,5 The skin pathergy reaction, another skin manifestation, has been recognized as a pathognomonic feature of Behçet’s disease, and it demonstrates the hyperreactivity of skin to nonspecific trauma . 6 In a positive pathergy test, pricking the skin with a sterile needle, with or without the injection of a small amount of saline, gives rise to a 1 to 2-mm papule usually surrounded by an erythematous halo. The papule may remain unchanged or transform into pustule which becomes maximum in size after 48 hours, and disappears within 4-5 days. A positive reaction is the development of an erythematous papulopustular skin reaction, similar to those ...