Sweet Syndrome

Definition

Rare dermatosis characterized by abrupt onset of painful skin plaques and nodules and associated fever, malaise and neutrophilia.

Clinical Features

Rare
Plaques and nodules:1–3
- uncommonly:
  - true bullae 4
- usually heal without scarring
- may be residual pigmentation:
  - attributed to hemosiderin
- variants:
  - raised annular lesions
  - palmoplantar pustulosis5,6
Location:
- face
- extremities
- less commonly, trunk
Onset:
- any age 7,8
Associated features:
- neutrophil leukocytosis 9,10
- may be:
  - episcleritis 10,11
  - intestinal involvement 12
  - leukemia:
    - 10–15% of cases13
    - usually acute myelomonocytic14–22
Many other associations 2,9,10,23–68

Pathogenesis

Etiology unknown
Assumed to represent immunological hypersensitivity reaction
Studies report:
- enhanced chemotaxis for neutrophils 69–71
- pathogenesis mediated through helper T-cell type 1 cytokines (IL-2, IFN-γ)72

Histopathology10,73

Mature neutrophils:
- dense infiltrate in:
  - upper half of dermis
  - may extend throughout dermis and into subcutis
  - epidermis usually spared
  - may simulate an incipient abscess because so dense in center
A neutrophil-poor variant is rare74
- common
Vessels:
- no true vasculitis or fibrinoid extravasation (Figs 1 and 2
  Fig. 1: Sweet syndrome. (H&E)
  Fig. 2: Sweet syndrome. There are numerous neutrophils surrounding a dermal blood vessel which is devoid of fibrin in its wall. (H&E)
  )
- often show endothelial swelling
Often:
- leukocytoclasis with formation of nuclear dust
- early forms of neutrophils, with bilobed nuclei:
  - may be mistaken for other cell types
May be a few eosinophils
In older lesions:
- lymphocytes:
  - usually:
    - perivascular
    - few in number
- sometimes prominent macrophages containing phagocytosed neutrophils in upper dermis
Papillary dermis:
- often marked edema (Fig. 3
  Fig. 3: Sweet syndrome with marked subepidermal edema. The underlying dermis contains a heavy infiltrate of neutrophils. (H&E)
  ):
  - may lead to subepidermal vesiculation
- delicate strands of dermal collagen usually stretch across the pseudobullous space10
- may be dilated vessels and extravasated red blood cells

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Sweet Syndrome

Sometimes features of atypical pyoderma gangrenosum:
- Sweet syndrome and pyoderma gangrenosum may be at opposite ends of spectrum of one process75–78
Erythema elevatum diutinum:79
- similar appearance except no fibrinoid material in Sweet syndrome
Granuloma faciale:
- more eosinophils, even in early lesions
- well-defined grenz zone

References

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3 Bulengo-Ransby SM, Brown MD, Dubin HV, et al.. Sweet's syndrome presenting as an unusual periorbital eruption. J Am Acad Dermatol. 1991;24:140–141.

4 Ahn S-J, Choi J-H, Sung K-J, et al.. Sweet's syndrome presenting with lesions resembling eruptive xanthoma. Br J Dermatol. 2000;143:449–450.

5 Christensen OB, Holst R, Svensson A. Chronic recurrent annular neutrophilic dermatosis. An entity?. Acta Derm Venereol. 1989;69:415–418.

6 Sommer S, Wilkinson SM, Merchant WJ, Goulden V. Sweet's syndrome presenting as palmoplantar pustulosis. J Am Acad Dermatol. 2000;42:332–334.

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53 Rubegni P, Marano MR, De Aloe G, et al.. Sweet's syndrome and Chlamydia pneumoniae infection. J Am Acad Dermatol. 2001;44:862–864.

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63 Cobb MW. Furosemide-induced eruption simulating Sweet's syndrome. J Am Acad Dermatol. 1989;21:339–343.

64 Thibault M-J, Billick RC, Srolovitz H. Minocycline-induced Sweet's syndrome. J Am Acad Dermatol. 1992;27:801–804.

65 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;34:918–923.

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69 Kaplan SS, Wechsler HL, Basford RE, et al.. Increased plasma chemoattractant in Sweet's syndrome. J Am Acad Dermatol. 1985;12:1013–1021.

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75 Caughman W, Stern R, Haynes H. Neutrophilic dermatosis of myeloproliferative disorders. J Am Acad Dermatol. 1983;9:751–758.

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Last updated: 30 Dec 2006