Vascular Inflammation (Vasculitis) in Sweet Syndrome

Abstract

Background: Sweet syndrome is characterized by painful, erythematous plaques of rapid onset accompanied by fever. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. We hypothesized that vasculitis can occur in Sweet syndrome and that it represents an epiphenomenon rather than a primary immune-mediated process.Design: Skin biopsy specimens from patients with Sweet syndrome were reviewed to determine the prevalence of vasculitis. The clinicopathologic features of cases with vasculitis were evaluated for statistically significant associations. Specimens with vasculitis underwent immunofluorescence staining.Setting: University department of dermatology, university hospital, and private practice.Patients: Medical records and biopsy specimens of 21 patients meeting diagnostic criteria for Sweet syndrome were reviewed. Interventions: None.Results: The prevalence of vasculitis was 29% (6 of 21 patients). There was a significant association of vasculitis with lesions of longer duration (P=.02). Vascular immunoglobulin and complement could not be demonstrated in cases of Sweet syndrome with vasculitis.Conclusions: Vasculitis is not a primary, immunemediated process in Sweet syndrome but occurs secondary to noxious products released from neutrophils. Blood vessels in lesions of longer duration are more likely to develop vasculitis than those of shorter duration because of prolonged exposure to noxious metabolites. Vasculitis does not exclude a diagnosis of Sweet syndrome.

The Relationship Between Neutrophilic Dermatosis of the Dorsal Hands and Sweet Syndrome

“…This agrees with 2 histologic series that identified leukocytoclastic vasculitis or vasculitislike changes in 18% to 30% of cases of typical Sweet syndrome. 23, 24 Malone et al 23 reviewed the presence of vasculitis in Sweet syndrome in relation to the timing of the biopsy and found that vasculitis correlated with lesions of longer duration. Indeed, 1 of our patients (case 8) had an initial biopsy result negative for vasculitis, then a subsequent positive biopsy result, suggesting a difference in lesion timing or sampling rather than a fundamental change in the disease process.…”

Elevated Serum Granulocyte Colony-Stimulating Factor Levels in Patients With Active Phase of Sweet Syndrome and Patients With Active Behçet Disease

“…Twelve cases satisfied the diagnostic criteria for SS. 18 Sixteen patients presented with BD, diagnosed according to the criteria proposed by the International Study Group for Behçet's Disease. 19,20 All patients were further divided according to either active or inactive state of the disease.…”

Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases

“…Histological examination revealed lymphocytic infiltration. Although this histological finding differs from Sweet's syndrome, vasculitis may be secondary to immunemediated processes in this disease [45]. This is also true for evaluation of the patients with cardiovascular diseases with aortitis, where histologic findings showed both lymphocytic and polymorphonuclear infiltration [11,28].…”