Atypical presentation of sweet syndrome with oral aphtheform ulceration and abdominal aortic aneurysm

Sweet syndrome is a relatively rare neutrophilic dermatosis characterized by fever, painful skin lesions with systemic involvement. It can occur secondary to multiple underlying aetiological factors. This entity can overlap with other neutrophilic dermatoses such as Behcet’s disease.
The patient is a fifty-two-year-old previously well male presented with classic sweet syndrome lesions with systemic unwellness associated with two painful aphtheform ulcerations in buccal mucosa which is relatively a rare presentation of this condition. Further evaluation revealed an incidental finding of an asymptomatic abdominal aortic aneurysm without any significant metabolic risk factors. His pathergy test and HLA B51 were negative. He did not have any other evidence of an underlying condition including haematological or solid organ malignancies, any culprit drugs, recent infections, rheumatoid arthritis or criteria to suggest Behcet’s disease. He gave a dramatic improvement for oral steroids and colchicine.
Though he does not fulfil the criteria for Behcet’s disease at the moment, being a well-known entity which overlaps with sweet syndrome, these two features of aphtheform oral ulcerations and vascular involvement in the form of abdominal aortic aneurysm could be part of evolving Behcet’s disease and he should be longitudinally followed up for further development of symptoms.