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Vascular Inflammation (Vasculitis) in Sweet Syndrome
A Clinicopathologic Study of 28 Biopsy Specimens From 21 Patients
Janine C. Malone, MD;
Stephen P. Slone, MD;
Lisa A. Wills-Frank, MD;
Paul K. Fearneyhough, MD;
Sheron C. Lear, HT(ASCP), HTL, QIHC;
L. Jane Goldsmith, PhD;
Antoinette F. Hood, MD;
Jeffrey P. Callen, MD
Arch Dermatol. 2002;138:345-349.
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, immune-mediated 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.
From the Department of Pathology and Laboratory Medicine (Drs Malone,
Slone, Wills-Frank, and Fearneyhough and Ms Lear), Division of Dermatology,
Department of Medicine (Drs Fearneyhough and Callen), and Department of Family
and Community Medicine (Dr Goldsmith), University of Louisville School of
Medicine, Louisville, Ky; and Division of Dermatopathology, Department of
Pathology, Indiana University School of Medicine, Indianapolis (Drs Malone
and Hood).
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