Histiocytic necrotizing lymphadenitis or Kikuchi disease is a rare entity; furthermore, its association with systemic lupus erythematosus (SLE) was only described in 11 patients in seven reports in the world literature. As the disease affects young women and manifests as a systemic illness associated with lymphadenopathy (usually cervical), the importance of continued follow-up to see whether it remains a self-limiting process or evolves into systemic lupus erythematosus should be emphasized. We present the clinical and histopathological data of eight patients with Kikuchi disease diagnosed in one tertiary care centre, two of whom evolved into SLE. White blood count was normal in all except the two patients who evolved into SLE; erythrocyte sedimentation rate was elevated in all patients tested. Kikuchi disease could be misdiagnosed as non-Hodgkin's lymphoma or tuberculous lymphadenitis; furthermore, awareness of its association with SLE is emphasized.
Forty-six patients with Behcet's disease were studied. Of those, 37 were Saudi and 9 were non-Saudi Arabs. Male-to-female ratio was 4.1:1. One hundred percent had mouth ulcers, 91% genital ulcers, 65% ocular involvement, 61% skin lesions, 59% joint involvement (nonerosive), 35% central nervous system manifestations, 26% gastrointestinal involvement, 26% vascular lesions, 11% psychiatric problems, and 11% pulmonary involvement. Only one patient had renal involvement which was documented by biopsy. In 72% of the patients, HLA-B5(51) was positive.
An 18-year-old man presented with a history of oral sores and presence of high fever, scrotal ulcerations and haemoptysis. Multiple mural cardiac masses were present in the right atrium, right ventricle and left ventricle. Furthermore, pulmonary vasculitis with aneurysm formation and venous thrombosis involving the superior sagittal sinus and right transverse sinus were found, and the diagnosis was made of (incomplete) Behçet's disease. While receiving anticoagulation and later, treatment with prednisone and cyclophosphamide, the cardiac thrombi gradually disappeared. We stress the importance of early echocardiography to evaluate cardiac abnormalities in Behçet's disease.
Scand J Rheumatol Downloaded from informahealthcare.com by Thomas Jefferson University on 12/26/14 For personal use only. Scand J Rheumatol Downloaded from informahealthcare.com by Thomas Jefferson University on 12/26/14 For personal use only.
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