kemia, followed by solid tumors of
the genitourinary and gastrointestinal
tract.1-4 The cutaneous manifestations
include eruption with papules that
can enlarge to form plaques. Pustu-lation and vesicles can also occur.
These lesions are asymmetric and
predominantly found on the upper
extremities, face, and neck and are
often described as feeling tender. The
patient may report hypersensitivity
within the affected area. Although
Sweet syndrome predominantly affects the skin, extracutaneous involvement of the musculoskeletal
system, gastrointestinal tract, heart,
and kidneys have been reported.1-3
The diagnosis of Sweet syndrome
is made by fulfilling both of the following major criteria: (1) an abrupt
onset of plaques or nodules that are
tender; and ( 2) histology of the lesions that include predominantly
neutrophilic infiltration of the dermis
in the absence of vasculitis.1, 5
In addition, 4 signs are consid-
ered minor criteria, and 2 are re-
quired for diagnosis. These include:
(1) preceding infection of the re-
spiratory or gastrointestinal tract,
or underlying cancer; ( 2) fever or
general malaise; ( 3) good response
to systemic corticosteroids; and ( 4)
abnormalities of laboratory values,
which can include elevated erythro-
cyte sedimentation rate (ESR), CRP,
segmented neutrophils in a periph-
eral blood smear, or leukocytosis.
Three of the 4 laboratory abnormal-
ities are required.
Although the patient fulfilled the
major criteria, she met only 1 minor
criterion. A CRP test was obtained;
however, an ESR was not obtained
during hospital admission. Since the
patient responded well to steroids
with significant reduction in lesions
and other symptoms, it was very
strongly suggestive of Sweet syn-
Treatment of Sweet syndrome
consists of daily systemic steroids
starting at 1 mg/kg and tapered over
4 to 6 weeks. Treatment generally
results in complete resolution of le-
sions. Extracutaneous symptoms also
respond well to steroid therapy. How-
ever, if an underlying malignancy
is present, the symptoms may not
respond to steroids, and treatment
should be focused on the malignancy.
Other treatments include indo-
methacin, colchicine, potassium
iodide, dapsone, and intravenous im-
munoglobulin, which can be used in
the long-term management of the dis-
order. The proposed mechanism of
action is thought to inhibit neutrophil
chemotaxis. Several other drugs have
also been used with good success.
The patient responded well to
steroids. A serum workup revealed
abnormalities in CRP, alkaline phos-
phatase, creatinine, and arthralgias
that have been described with this
disorder. Biopsy pathology showed
classic histological features of Sweet
syndrome. After hospitalization, the
patient completed a prolonged steroid
taper, and her lesions resolved 1 week
after her hospital stay. Given Sweet
syndrome has an association with a
variety of malignancies, the patient is
currently undergoing the necessary
screening examinations, including a
mammography, colonoscopy, and an
evaluation of her blood work. ●
The authors report no actual or potential conflicts of interest with regard to
The opinions expressed herein are those
of the authors and do not necessarily
reflect those of Federal Practitioner,
Quadrant HealthCom Inc., a division
of Frontline Medical Communications
Inc., the U.S. Government, or any of its
agencies. This article may discuss unlabeled or investigational use of certain
drugs. Please review complete prescribing information for specific drugs or
drug combinations—including indications, contraindications, warnings, and
adverse effects—before administering
pharmacologic therapy to patients.
1. Sweet RD. An acute febrile neutrophilic dermatosis.
Br J Dermatol. 1964;76:349-356.
2. Kemmet D, Hunter JA. Sweet’s syndrome: A clinico-pathological review of twenty-nine cases. J Am Acad
Dermatol. 1990; 23( 3, pt 1):503-507.
3. Cohen PR. Sweet’s syndrome—A comprehensive
review of an acute febrile neutrophilic dermatosis.
Orphanet J Rare Dis. 2007;2: 34.
4. Neoh CY, Tan AW, Ng SK. Sweet’s syndrome: A
spectrum of unusual clinical presentations and associations. Br J Dermatol. 2007;156( 3):480-485.
5. Habif TP. Clinical Dermatology: A Color Guide to
Diagnosis and Therapy. 5th ed. Edinburg, Scotland:
Figure 2. Similar lesions located on forearm.