Subject(s)
Anti-Inflammatory Agents/administration & dosage , Methylprednisolone/administration & dosage , Retinal Vasculitis/drug therapy , Retinitis/drug therapy , Vasculitis/drug therapy , Administration, Intravenous , Adult , Female , Glucocorticoids/administration & dosage , Humans , Intravitreal Injections , Retinal Vasculitis/complications , Retinitis/complications , Syndrome , Vasculitis/complicationsABSTRACT
We report the case of a 57-year-old man, presenting with bilateral panuveitis, bilateral sacroiliitis, intermittent pyrexia and a pulmonary nodule. The patient had been under immunosuppressive treatment for 2 years for Behçet's disease. However, he did not fulfill the diagnostic criteria of Behçet's disease. Blood analysis showed a very high C reactive protein (CRP at 34 mg/dl). In view of severe intra-ocular inflammation, the anterior chamber was punctured. Polymerase chain reaction (PCR) on the aqueous humour and on the blood revealed the presence of Tropheryma whippelii DNA, an agent responsible for Whipple's disease. The patient was treated with ceftriaxone followed by trimethoprim-sulfamethoxazol for 1 year with good clinical and biological evolution. This case illustrates the difficulty to diagnose an atypical Whipple's disease. In cases of uveitis with atypical signs and/or not responding to the treatment, the internist must consider to perform an analysis of the ocular fluids.
Subject(s)
Whipple Disease/diagnosis , Aqueous Humor/microbiology , Biopsy , C-Reactive Protein/metabolism , DNA, Bacterial/analysis , Diagnosis, Differential , Duodenum/pathology , Humans , Male , Middle Aged , Polymerase Chain Reaction , Tropheryma/genetics , Tropheryma/isolation & purification , Whipple Disease/metabolismABSTRACT
This case report describes the evolution of a mycosis fungoides into a Sézary syndrome. The originality of the case consists in the appearance of ascitis with Sézary cells during the leukemic phase. It is the second report of a such case. Mycosis fungoides and its leukemic variant, the Sézary syndrome, are primary cutaneous T-cell lymphomas. Their incidence is low. The treatments are topical in the early stages and systemic during the advanced stages. New immunomodulating treatments are in development. The existing therapeutic agents unfortunately do not improve the prognosis of the disease today.