Sweet's syndrome

This condition was originally described by Dr. Robert Douglas Sweet in 1964

• an acute febrile neutrophilic dermatosis
  • characterized by fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, with prompt improvement after the initiation of treatment
    • lesions have a transparent, vesicle-like appearance secondary to the pronounced oedema in the upper dermis (pseudovesicular appearance)
    • clinical presentation is with acute, tender, erythematous plaques, pseudovesicles and, occasionally, blisters with an annular or arciform pattern
      • rash occurs on the head, neck, legs, and arms, particularly the back of the hands and fingers
        • trunk is rarely involved
          
    • histological findings of dense neutrophilic infiltrate without any evidence of primary vasculitis
      
      
• three main clinical types which are described include: Classical or idiopathic SS, malignancy-associated or paraneoplastic SS, and drug-induced SS
  • Classical or idiopathic Sweet's syndrome may be associated with infection (upper respiratory tract or gastrointestinal tract), inflammatory bowel disease, or pregnancy
    
    
• recurrent episodes of Sweet's syndrome occur in one-third to two-thirds of patients
  
  
• Sweet's syndrome can also present as a paraneoplastic syndrome (most commonly related to acute myelogenous leukaemia) or as a medication-related disorder (most commonly after treatment with granulocyte-colony stimulating factor therapy)
  • approximately 70% of cases are idiopathic and the paraneoplastic form is present in 10-20% associated predominantly with hematological malignancies such as acute myelogenous leukemia, myelodysplastic syndromes and lymphoma (2,3,4)
    
    
• drug-induced SS
  • pharmacovigilance signals have been observed with use of colony-stimulating factors, immunosuppressants, antineoplastics and antibiotics (6)

Improvement in patients with malignancy-associated Sweet's syndrome or drug-induced Sweet's syndrome may occur following successful treatment of the cancer or discontinuation of a causative medication. The therapeutic mainstay for Sweet's syndrome is systemic corticosteroids

• dapsone has been used as either monotherapy or in combination therapy (4,5) Other agents used in the management of this condition include potassium iodide, colchicine, Indomethacin, clofazimine, cyclosporine, and antibiotics
• corticosteroids continue to be efficacious first-line therapy for the majority of patients (7)

Reference:

• Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56.
• Cohen PR, Kurzrock R. Sweet's syndrome and cancer. Clin Dermatol 1993;11:149-57.
• Payda S, Sahin B, Seyrek E, Soylu M, Gonlusen G, Acar A, et al . Sweet's syndrome associated with G-CSF. Br J Haematol 1993;85:191-2.
• Kemmett D, Hunter JA. Sweet's syndrome: A clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990;23:503-7.
• Aram H. Acute febrile neutrophilic dermatosis (Sweet's syndrome): Response to dapsone. Arch Dermatol 1984;120:245-7
• Martin, S, Trenque, T, Herlem, E, Boulay, C, Pizzoglio, V, Azzouz, B. Drug-induced Sweet’s syndrome: a case/non-case study in the French pharmacovigilance database. Br J Clin Pharmacol. 2023. Accepted Author Manuscript. https://doi.org/10.1111/bcp.15873
• Joshi TP, Friske SK, Hsiou DA, Duvic M. New Practical Aspects of Sweet Syndrome. Am J Clin Dermatol. 2022 May;23(3):301-318. doi: 10.1007/s40257-022-00673-4. Epub 2022 Feb 14. PMID: 35157247; PMCID: PMC8853033