Association of Sweet's Syndrome and Behçet's Disease: Is It Possible? A Case Report

Eur J Case Rep Intern Med. 2023 Sep 19;10(10):004073. doi: 10.12890/2023_004073. eCollection 2023.

Abstract

Introduction: Behçet's disease is a systemic vasculitis characterized by a large clinical polymorphism with a particular frequency of cutaneous signs. Sweet's syndrome is a neutrophilic dermatosis marked by the sudden appearance of painful skin lesions in the form of erythematous papules, nodules or plaques. This syndrome is associated with high fever, neutrophilia and histologically a diffuse infiltrate of neutrophils in the dermis.

Observation: We report the case of a 43-year-old patient followed for Behçet's disease, who developed cutaneous plaques of neutrophilic dermatosis of both upper limbs. The clinical and biological picture was in favor of Sweet's syndrome.

Conclusion: The coexistence of Sweet's syndrome and Behçet's disease is already reported in the literature. The association is however very rare given the differences in the clinical and pathogenic features between the two conditions.

Learning points: The appearance of neutrophilic dermatosis during a skin flare-up of Behçet's disease alerted us to a possible link between Sweet's syndrome and Behçet's disease.The morphology of the skin lesions associated with these pathologies is heterogeneous, making diagnosis sometimes difficult.Cases reported in the literature concerning the association between Sweet's syndrome and Behçet's disease are rare.

Keywords: Behçet’s disease; Sweet’s syndrome; association; pathogenesis.