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Integumentary system ORGAN SYSTEMS

Febrile neutrophilic dermatosis “Sweet’s syndrome”

Sweet’s syndrome (SS), or acute febrile neutrophilic dermatosis is a skin disease characterized by the sudden onset of fever, leukocytosis, acute, tender, red papules & plaques and a papillary dermal infiltrate of neutrophils.

Sweet’s syndrome (SS), or acute febrile neutrophilic dermatosis is a skin disease characterized by the sudden onset of fever, leukocytosis, acute, tender, red papules & plaques and a papillary dermal infiltrate of neutrophils.

History

The syndrome was first described in 1964 by Robert Douglas Sweet. It was also known as Gomm-Button disease in honour of the first two patients Sweet diagnosed with the condition.


Aetiology

  • Classical/idiopathic SS
  • Malignancy-associated SS (paraneoplastic syndrome)
  • Drug-induced SS

Clinical features

  • Acute, tender, erythematous plaques, nodes, pseudovesicles and, occasionally, blisters
    • Pattern: Annular/arciform
    • Location: Head, neck, legs, and arms, particularly the back of the hands and fingers. The trunk is rarely involved.
sweet-syndrome_skin
(a, b, and c). Tender, red Sweet’s syndrome lesions in a woman with the classical form of the dermatosis. A closer view of the Sweet’s syndrome lesions from the woman in Figure 1. The skin lesions improved rapidly after corticosteroid therapy was initiated. There is an erythematous plaque, 5 cm in diameter, with a pseudovesicular appearance on the left shoulder of the patient (a). A nodular lesion, 1 cm in diameter, is present on the lateral left arm (b). Painful, erythematous, pseudovesicular plaques of acute febrile neutrophilic dermatosis are present on the left hand (c). | Cohen PR: Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007 Jul 26;2:34. PMID 17655751, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=4054245
640px-sweet27s_syndrome_crohn27s_disease
Pustular lesions with central necrosis on the left leg of a patient with Sweet’s syndrome associated with Crohn’s disease. | Nadia M Mustafa, Mark Lavizzo. – Sweet’s syndrome in a patient with Crohn’s disease: a case report. Journal of Medical Case Reports 2008, 2:221. doi:10.1186/1752-1947-2-221, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=5958955

Associated features:

  • Fever (50% cases)
  • Arthralgia/arthritis (62% cases)
  • Eye involvement: conjunctivitis/iridocyclitis (38% cases)
  • Oral aphthae (13% cases)

Diagnosis

Blood panel:

  • Moderate neutrophilia (< 50% cases)
  • ↑ ESR (> 30 mm/h) (90% cases)
  • Slight ↑ in alkaline phosphatase (83% cases)

Histopathology:

  • Papillary and mid-dermal mixed infiltrate of polymorphonuclear leukocytes with nuclear fragmentation and histiocytic cells.
    • Infiltrate is predominantly perivascular with endothelial-cell swelling in some vessels, but vasculitic changes (blood clots; deposition of fibrin, complement, or immunoglobulins within the vessel walls; RBC extravasation; inflammatory infiltration of vascular walls) are absent in early lesions.
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Punch biopsy of a skin lesion showing neutrophilic infiltration in the dermis, with no evidence of vasculitis | Nadia M Mustafa, Mark Lavizzo. – Sweet’s syndrome in a patient with Crohn’s disease: a case report. Journal of Medical Case Reports 2008, 2:221. doi:10.1186/1752-1947-2-221, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=5959132

Differential diagnosis:

  • Pyoderma gangrenosum
  • Infection
  • Erythema multiforme
  • Adverse drug reactions
  • Urticaria

Management

  • Systemic corticosteroids (prednisone) (GOLD STANDARD)
    • Tapered within 2-6 weeks to 0
  • Indomethacin (2nd-line treatment, or corticosteroid contraindication)
    • 150 mg/day (1st week)
    • 100 mg/day (2 weeks)
  • Alternative drugs:
    • Dapsone, doxycycline, clofazimine, and cyclosporine

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