Atypical Behçet's syndrome in a patient with myelodysplastic syndrome

We report the case of a 67-year-old man with myelodysplastic syndrome (MDS), who presented with fever, painless penile and groin ulcers, as well as oral and esophageal ulcerations, all of which were exquisitely responsive to corticosteroids. Some cases of Behçet's syndrome and MDS have been reported in association with trisomy 8 and HLA B51, with the pathology varying from vasculitis to acute neutrophilic inflammation. Our patient with orogenital ulcers had neither trisomy 8 nor HLA B51. Also lacking were other features typical of Behçet's syndrome, such as uveitis, vasculitis, and central nervous system abnormalities. However, his response to corticosteroids on the initial presentation and subsequent episodes was dramatic. Esophageal ulcerations have not been described in Behçet's syndrome. In this respect, our patient was unique.