Diagnostic criteria for Sweet syndrome

Classical*

1. Abrupt onset of painful erythematous plaques or nodules

2. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis

3. Pyrexia >38°C

4. Association with an underlying hematologic or visceral malignancy, inflammatory disease, or pregnancy, or preceded by an upper respiratory or gastrointestinal infection or vaccination

5. Excellent response to treatment with systemic corticosteroids or potassium iodide

6. Abnormal laboratory values at presentation (three of four): erythrocyte sedimentation rate >20 mm/hr; positive C-reactive protein; >8,000 leukocytes; >70 percent neutrophils

Drug-induced^¶

A. Abrupt onset of painful erythematous plaques or nodules

B. Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis

C. Pyrexia >38°C

D. Temporal relationship between drug ingestion and clinical presentation, or temporally-related recurrence after oral challenge

E. Temporally-related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids

The patients with malignancy-associated Sweet syndrome are included with the patients with classical Sweet syndrome in this list of diagnostic criteria.

* The presence of both major criteria (1 and 2), and two of the four minor classical Sweet syndrome.
¶ All five criteria (A, B, C, D, and E) are required for the diagnosis of drug-induced Sweet syndrome.

Original figure modified for this publication. Walker DC, Cohen PR. Trimethoprim-sulfamethoxazole-associated acute febrile neutrophilic dermatosis: case report and review of drug-induced Sweet's syndrome. J Am Acad Dermatol 1996; 34:918. Table used with the permission for Elsevier Inc. All rights reserved.

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Diagnostic criteria for Sweet syndrome