Entero-Behçet disease associated with sweet syndrome: A case report

Sweet syndrome is an acute neutrophilic dermatosis that may be associated with systemic inflammatory diseases. Behçet disease is a multisystem vasculitis whose gastrointestinal involvement, referred to as entero-Behçet disease, may be severe. The association between these two entities remains exceptionally rare.
We report the case of a 32-year-old woman diagnosed with entero-Behçet disease based on bipolar aphthosis, a positive pathergy test, and characteristic intestinal ulcerations observed on endoscopy. One month later, she developed an extensive cutaneous eruption consisting of infiltrated erythematous plaques, vesiculopustular lesions, and bullae, associated with biological inflammatory syndrome. Histopathological examination of the skin biopsy revealed a dense neutrophilic infiltrate without evidence of vasculitis, consistent with Sweet syndrome. Treatment with systemic corticosteroids combined with colchicine resulted in complete regression within 20 days.
This case highlights a rare association likely related to a shared neutrophilic hyperactivation pathway and underscores the importance of early diagnosis in order to optimize therapeutic management.