Lymphoma mimicking Beh?et's disease:case report and literature review / 中华风湿病学杂志

ABSTRACT

Objective To study the clinical char acteristics and outcome of two lymphoma patients mimicking Beh?et's disease. Methods Lymphoma was diagnosed in two patients mimicking Beh?et's disease referred to our Department in 2015. A search on published similar cases in Chinese database and the Pubmed was also performed and then analyzed. Results Eight patients were indentified in this pooled analysis, six of which were non-Hodgkin lymphoma (NHL). All of the eight cases presented with cutaneous lesion, seven cases with fever, seven cases with oral ulceration and six cases with orogenital ulceration, respectively. Ocular involvement was present in four of the eight cases, two were with a positive pathergy test. Among feverish patients, six were moderate or high fever, four were high fever, one was low-grade fever. Neutropenia was found in four patients, and lymphocytoponia in four of five patients with detailed data. All patients fulfilled the 2014 International Criteria for Beh?et's Disease (ICBD) with an average score of (5.8 ±1.5), ranging from 4 to 8. Survival period ranged from one month to 36 months, with an average of 8 months. Conclusion For patients diagnosed as Beh?et's disease are finally diagnosed as lymphoma. For patients with Beh?et's disease present-ation but also present with mediate to high fever, atypical deepseated ulcer, neutropenia or lymphocytoponia, malignancy especially lymphoma should be investigated.