Transcript Sweet`s Syndrome - UNC School of Medicine

Sweet’s Syndrome
Allison Dupont
AM Report
1/17/06
Definition

Sweet’s syndrome (acute febrile
neutrophilic dermatosis) is characterized
by:




Fever
Peripheral neutrophilia
Painful red skin papules, nodules and/or
plaques
Neutrophilic infiltration of skin (particularly
the dermis)
Clinical Presentation



Fever (>38˚C) can be intermittent and may precede skin
manifestations by days to weeks.
Systemic symptoms may include headache, myalgia,
arthralgia, and general malaise.
Cutaneous lesions consist of erythematous to violaceous
tender papules which may coalesce to form plaques.



The plaques are not pruritic.
Most often found on the face, neck and upper extremities
(especially the dorsum of the hands), but can occur anywhere.
Lesions on the lower extremities may resemble erythema
nodosum.
Clinical Presentation


Oral ulcers (more common in patients
with Sweet’s syndrome and a hematologic
malignancy.
Ocular involvement (uncommon in
malignancy-associated and drug-induced
Sweet’s syndrome).

Conjunctivitis, episcleritis
Clinical Presentation

Involvement of internal organs may occur
leading to:




Alveolitis
Sterile osteomyelitis
Involvement of liver, pancreas, and/or
kidneys
Neurologic and psychiatric changes
Laboratory Findings



Lab findings are nonspecific.
Majority will have peripheral neutrophilia.
Other possible lab abnormalities include:




Elevated sedimentation rate
Elevated C-reactive protein
Leukocytosis
Consider evaluation of hepatic and renal
function.
Pathology



Sweet’s syndrome characteristically
involves dense neutrophilic infiltration of
the dermis +/- dermal edema.
Neutrophil karyorrhexis is commonly
seen.
There is no involvement of the vasculature
of the skin and no necrosis (in contast to
pyoderma gangrenosum).
Diagnosis
Major Criteria

Abrupt onset of painful erythematous
plaques or nodules.

Histopathologic evidence of a dense
dermal neutrophilic infiltrate without
vasculitis.
Minor Criteria

Fever (>38˚C)

Association with an underlying
hematological/visceral malignancy,
inflammatory disease, or pregnancy or
preceded by an upper respiratory or
GI infection or vaccination.

Excellent response to treatment with
systemic corticosteroids.

Peripheral neutrophilia (>70%
neutrophils)
Associated Conditions



Sweet’s syndrome is associated with an
underlying disease or condition in up to 50% of
patients.
Sweet’s syndrome may be the presenting sign
and the underlying disease may not become
apparent for several years after Sweet’s
syndrome occurs.
There is a female predominance except in the
case of malignancy-associated Sweet’s
syndrome.
Associated Conditions
1.
Malignancy



2.
Approximately 21% of patients with Sweet’s
syndrome have a malignancy.
15% hematological (most commonly AML)
6% solid tumors (most commonly carcinomas of
the GU tract, GI tract, or breast)
Infections


Mostly of the upper respiratory or GI tract
Streptococcus, mycobacterium, Yersinia,
Salmonella, Shigella
Associated Conditions
3.
Inflammatory bowel disease

4.
5.
Sweet’s syndrome may occur alone or in combination with
pyoderma gangrenosum.
Pregnancy
Other conditions with a possible association:
-Sarcoidosis
-Rheumatoid arthritis
-Thyroid disease (Grave’s disease and Hashimoto’s thyroiditis)
Drug-induced Sweet’s syndrome



Criteria slightly different than classical
syndrome.
Temporal relationship between drug
ingestion/injection and clinical
presentation.
Resolution of lesions/symptoms after
withdrawal of drug or treatment with
corticosteroids.
Drug-induced Sweet’s syndrome


G-CSF is responsible for the majority of
cases.
Other possible causes: furosemide,
lithium, hydralazine, trimethoprimsulfamethoxazole, and oral contraceptives.
Pathogenesis




Etiology of Sweet’s syndrome is unknown.
Presumed to be due to a hypersensitivity
reaction to an eliciting antigen which
leads to stimulation of cytokine release.
Cytokines precipitate neutrophil
activation and infiltration.
Response to treatment with corticosteroids
supports this etiology.
Pathogenesis

The source of the eliciting antigen may be
diverse, including bacterial, viral or
tumoral antigens.
Treatment

Gold standard: Systemic corticosteroids
Start at 1 mg/kg/day prednisone with long
taper (4-6 weeks) to 10 mg/day.
 Many patients require several months of
10-30 mg/day to suppress recurrences.


Localized Sweet’s syndrome can
sometimes be treated with high-potency
topical corticosteroids.
Treatment
Other first-line agents include:
1.
Oral potassium iodide
-Systemic symptoms resolve in 1-2 days.
-Dermatitis resolves in 3-5 days.
2.
Colchicine
-Systemic symptoms resolve in 2-3 days.
-Dermatitis resolves in 2-5 days.
Alternative therapies




Indomethacin
Clofazimine
Cyclosporine
Dapsone
References
Burall, BarbaraM .D. “Sweet’s sy
n drome (acute febrilen eutrophilic dermatosis)”. Dermatology O
n lin e
Journ al 5(1):8.
Cohe
n , Philip R.M D, Kurzrock, RazelleM D. “Sweet’s syn drome revisited: a review of disease
co
n cepts”. In tern atio
n al Journ al of Dermatology. Volume 42, Issue 10. October 2003.
Cohe
n , Philip R. “Sweet’s sy
n drome”. Orpha
n et. October
2003.
Federma
n et al. “Cuta
n eous ma
n ifestatio
n s of malig
n a
n cy”. PostgraduateM edicin e O
n lin e. Ja
n uary
2005.
Joe, Edwin K.M D. “Sweet’s sy
n drome”. Dermatology O
n lin e Journ al 9(4):28.
Moschella, Samuel L.M D. “Neutrophilic dermatoses”. UpToDate.
“Sweet’s Syn drome”. Dermis.n et.