Journal of the

American Academy of

DERMATOLOGY VOLUME 31 NUMBER 4 OCTOBER 1994

CONTINUING MEDICAL EDUCATION

Sweet's syndrome (acute febrile neutrophilic

dermatosis)
Peter von den Driesch, MD Erlangen, Germany

Acute febrileneutrophilic dermatosis, first described in 1964by Robert Douglas Sweet, has
beentermed Sweet's syndrome. Classic Sweet'ssyndromeoccursin middle-aged women af-
ter a nonspecific infection of the respiratory or gastrointestinal tract. Raised erythematous
plaques with pseudoblistering and occasionally pustules occur on the face, neck, chest, and
extremities, accompanied by fever and general malaise. Involvement of the eyes, joints, and
oral mucosaas wellasinternalmanifestations ofSweet'ssyndromein the lung, liver, kidneys,
and central nervous system has been described. The disease is thought to be a hypersensitiv-
ity reaction. Parainflammatory (e.g., infections, autoimmune disorders, vaccination) and
paraneoplastic (e.g., hemoproliferative disorders, solidmalignanttumors) occurrenceis found
in approximately 25%of the casesand 2% are associated withpregnancy.Sweet's syndrome
responds rapidlyto systemic therapywithcorticosteroids but recursin about 25% of the cases.
Alternative treatment modalities (e.g., potassium iodide, colchicine, dapsone, clofazimine,
cyclosporine) have also been used.This article presents data from 38 patients with Sweet's
syndromeand reviews its epidemiology, clinical spectrum, histologic features, laboratory re-
sults, differential diagnosis, pathogenic mechanisms, associated diseases, and treatment. (J
AM ACAD DERMATOL 1994;31:535-56.)

Learning objective: At the conclusion of this learning activityparticipants shouldbe able to

outlinethe essential featuresof Sweet'ssyndrome, includingaspectsof epidemiology, histol-
ogy,diagnosis, and management of the condition.

The first description of Sweet's syndrome as a new
entity derived from the observation of eight female
patients seen in the General Hospital ofPlymouth by
Dr. Robert Douglas Sweet. I He termed the disease
acute febrile neutrophilic dermatosis because of the
acute onset of fever, leukocytosis, and erythematous
plaques infiltrated by neutrophils. Whittle et a1. 2 and
Crowet a1. 3 were the first who used the term Sweet's
The CME articlesare madepossible throughaneducational
grant from the Dermatological Division, Ortho Pharma-
ceuticalCorporation.
Supported by the Johannes and Frieda Marohn Foundation,
DRI-90.
Reprintrequests: P.von den Driesch, MD, Dept.ofDermatology, Uni-
versity ofErlangen-Nilmberg, Hartmannstr. 14,D-91 052Erlangen,
Germany.
Copyright @ 1994by the American Academyof Dermatology, Inc.
0190-9622/94 $3.00 +0 16/2/57249
syndrome in the title of their subsequent case
descriptions.
Between 1969 and 1974 many reports worldwide
confirmed the existence of this new entity (cited in
Harms"). More than 500 cases have been docu-
mented in the literature. Shapiro et a1. 5 first reported
the association of Sweet's syndrome with a solid
carcinoma, whereas Matta et a1. 6 first described the
occurrence during a course of a hemoproliferative
neoplasm. Klock and Oken 7 first observed a 10-
year-old child suffering from Sweet's syndrome. In
1986 Su and Liu'' proposed two major and four mi-
nor criteria for the diagnosis of Sweet's syndrome,
which have been widely accepted. The disease has
been the subject of several excellent reviews.P''?
Harms4 summarized the literature from 1964 to
1980 and Cohen and Kurzrock l 8-20 reviewed all
paraneoplastic cases.
535
 Journal of the American Academy of Dermatology
536 von den Driesch October 1994

patients
7.--------------------------,
6 --.-----." .._-

o
2 3 4 5 6 7 8 9 10 11 12
month
Fig. 1. Analysis of month of onset in 38 cases of Sweet's syndrome suggests seasonality in
occurrence of the disease.

Table I. Summary of findings in 38 patients with Sweet's syndrome

Duration of the study January 1985-August 1993
Sex 28 F/l0 M
Mean age F, 48 yr; M, 65 yr; all, 53 yr
Occurrence Classic, 17; idiopathic, 5; parainflammatory, 12;
paraneoplastic, 3; pregnancy (first trimester,
healthy child), 1
Associated findings Fever or subfebrile body temperature, 78%
Arthritis/arthralgia, 57%
Conjunctivitis/episcleritis, 33%
Associated parainflammatory conditions Crohn's disease (2 ptS),70 chronic rheumatoid ar-
thritis (2 pts), ulcerative colitis, Hashimoto's
thyroiditis, Sjogren's disease, yersiniosis,chole-
cystitis, pyelonephritis, vulvovaginal infec-
tion,S7 otitis media.t? tonsillitis-?
Associated neoplastic conditions Centroblastic non-Hodgkin's lymphoma of high
malignancy
Adenocarcinoma of the colon196
Adenocarcinoma of the prostate
Urticaria pigmentosa, stable disease
Therapy and follow-up Methylprednisolone in 37 pts, relapses after dis-
continuation in 9 pts, chronic relapsing course
in 6 pts, more than one episode of Sweet's syn-
drome after disease-free interval of 12 mo in
3 pts, cyclosporine in 1 pt, clofazimine in 6 pts
with chronic relapsing course, initial dose
200 mg/day for 4 wk, 100 mg/day for another
4 wk, almost complete remission in all pts, no
systemic treatment necessary afterwards
pts, Patients.

EPIDEMIOLOGY
Limited data are available about the incidence
and prevalence of Sweet's syndrome. The diagnosis
was made in 1 of 250 outpatients in Sri Lanka-' and
in 1 of 1200 outpatients in our department in South
Germany (Table I). In Scotland an annual incidence
of 2.7 cases per 106 population has been estimated.F
In Geneva, Switzerland, a highly variable annual
Journal of the American Academy of Dermatology
Volume 31, Number 4 von den Driescn 537

Patients
!
__ __ _L Li i
\L__----l- I
;! r---:1\.-.'-_

. J
I Ij_._•.__ I
j.. ._ '
50 I !
40

30

20
10

10-19 20-29 30-39 40-49 50-59 60-69 70-79 >80

Age
fa male patients • female patients g all patients
Fig. 2. Age distribution in 176 cases of Sweet's syndrome collected in seven
series." .22,24-27, this report

Table II. Findings in 174 patients with Sweet's syndrome in seven series in the literature
Mean age CLA/ID PI PN
No. of F/M (yr) Duration (No. of (No. of (No. of PRE (No.
Author(s) patients ratio (only M) (yr) patients) patients) patients) of patients)

Gunarwardena et a1. 21 18 17:1 49(61) 2 16 2

Smolle & Kresbach/" 18 16:2 52(44) ND 13 2 3
Delabie et a1.25 18 11:7 57 (65) 11 11 4 3
Kemmett & Hunter22 29 25:4 56 (55) 16 18 4 5 2
Butzmann26 24 20:4 47 (56) 10 23 1
Sitjas et alP 30 21:9 55 (56) 12 20 5 4 I
Current study 38 28:10 53 (65) 9 22 12 3 1
Totals 176 139:37 53 (59) 124 29 19 '4
3.7:1 (71%) (16%) (11%) (2%)
eLA/ID, Classicor idiopathicoccurrenceof Sweet's syndrome; F, female; M, male; ND, no data; PI, parainfiammatory occurrence of Sweet's syn-
drome; PN, paraneoplastic occurrence of Sweet'ssyndrome; PRE, pregnancy-associated occurrence of Sweet's syndrome.

incidence raised the possibility of an infectious
cause.23 However, whether the rapidly rising num-
ber of case reports is due to an increasing aware-
ness or incidence of Sweet's syndrome remains un-
clear.
Onset of the disease in our 38 cases clustered in
two peaks in spring and autumn (Fig. 1).
Data from 176 patients with Sweet's syndrome
from seven larger series21, 22, 24-27 (Table II) dem-
onstrate an overall female/male ratio is 3.7:1. The
largest and most recent series." however, revealed a
lower female predominance of 2.3-2.8: 1. The mean
age of the 176 patients was 52.6 years with a broad
incidence peak in the fourth to seventh decades (Fig .
2). The frequent occurrence in women between 30
and 49 years of age suggests a second incidence peak
for younger women.
Twenty-seven children with Sweet's syndrome
have been reported.?: 11, 16, 28·50 The male/female
ratio of these patients is about 1:1; one incidence
peak occurs in the first year of life and a second be-
tween 6 and 12 years of age.
Sweet's syndrome can be subdivided into four
broad groups, that is, classic/idiopathic, parain-
flammatory, and paraneoplastic, as well as occur-
rence during pregnancy. Seventy-one percent of the
176 cases collected in the seven series belonged to the
first group, 16% were parainfl.amrnatory, 11% pa-
raneoplastic, and 2% were pregnant. The rate of
paraneoplastic cases of up to 33% estimated in the
literature'! may in part be due to the tendency to
publish case reports with severe associated diseases.
CLINICAL SPECTRUM
The occurrence of multiple, painful, sharply de-
marcated, raised erythematous plaques on the face,
 Journal of the American Academy of Dermatology
538 von den Driescli October 1994

Fig. 3. Classic lesions of Sweet's syndrome. A and B, Occurrence in a 36-year-old woman

7 days after infection of upper respiratory tract. C and D, Idiopathic Sweet's syndrome in 71-
year-old man. E, Paraneoplastic Sweet's syndrome in 51-year-old woman with high-grade
centroblastic non-Hodgkin's lymphoma.

neck, upper chest, back, and extremities is the clin-
ical hallmark of Sweet's syndrome (Fig. 3). These
plaques are painful and burning but characteristi-
cally not pruritic. The size is normally a few centi-
meters, but large plaques up to lOX 20 ern in diam-
eter (Fig. 3, B) may develop. Fig. 4 summarizes the
localization oflesions seen in 15 consecutive patients
with Sweet's syndrome. The surface of these plaques
may exhibit a mamillated appearance with
pseudovesiculation, pseudopustulation, and pustules
(Fig. 5); in rare instances severe blistering may oc-
cur (Fig. 5, D). The plaques are red to blue-red,
sometimes with a faint central white-yellow discol-
oration leading to the impression of a target lesion
similar to erythema multiforme (EM). A subtype of
Sweet's syndrome with a single lesion or few lesions
Journal of the American Academy of Dermatology
Volume 31, Number 4
often manifests itself on the face- 11,52-55 and has
been termed localized Sweet's syndrome. Fever and
leukocytosis are often absent in these patients.
Lymph node involvementor swelling is not a reg-
ular feature of the disease but may be present in
some patients."
Compared with EM, oral manifestations are less
characteristic for Sweet's syndromeand occurin 3%
to 30% of patients.v- 56 Early pseudopustular le-
sions'" that closelyresemblethe pustular skin man-
ifestations evolve to aphthoid lesions 58 (Fig. 6, A).
The lips, the buccal mucosa, and the tongue are the
sites of predilection.
Genital lesions are rare.59-61 Behcet's syndrome,
in particular, has to be ruledout evenin patientswho
maybe clinically diagnosedas Sweet's syndrome.P
On the lower legs, lesions resembling erythema
nodosum (EN) are common (Fig. 6, B). These
lesions are almost indistinguishable from EN24,63-72
and have been observed in 30% of patients of a
Spanish series,27 in 17% of our patients, and in 12%
of 198 reported Japanese patients.56 However, pa-
tients with EN alone after or before an episode of
Sweet's syndrome with EN lesions have also been
reported.7o,73.76
Cutaneous pathergy, well-known in Behcet's dis-
ease or pyoderma gangrenosum, may also occur in
Sweet's syndrome29. 53,77 and was present in 8% of
our patients. In addition, skin lesions may be pro-
duced after trauma or UV irradiation suggesting a
Koebner phenomenon.P:"?
Subfebrile body temperature or fever occur in
48%22 to 83%21 of patients. These elevations may be
intermittent, especially in long-lasting untreated
forms.57
Arthralgias or arthritis occur in 33%to 62%22,24
of patients. Joint involvement manifests itself as
polyarthritis and affectshands, wrists,ankles, knees,
and shoulders.s- 69, 80-87 Eye involvement has been
reported in 6%to 72%21,24 of the patients. The data
from larger series22, this report indicate that it is to be
expected in about one third of patients. Conjunc-
tivitis and episc1eritis are the main features,2!,88-91
whereas uveitis,n limbal nodules." and inflamma-
tory glaucoma'" have been reported only occasion-
ally.
HISTOLOGIC FINDINGS
In Sweet'soriginalreport 1 and in a comprehensive
subsequent study by Jordaan." the histologic crite-
ria for Sweet's syndrome were defined. An infiltrate
consisting of mononuclear cells and numerous neu-
von den Driesch 539
Fig. 4. Localization of Sweet's syndrome lesions in 15
consecutive patients.
trophils with leukocytoclasis, a marked vasodilation
and swelling of the vascular endothelium with mod-
erate erythrocyte extravasation, and prominent
edema of the upper corium frequently leading to the
formation of vesicles or bullae are the main charac-
teristics (Fig. 7). The inflammatory cells exhibit a
bandlike infiltration throughout the papillary der-
mis.Perivascularmononuclear infiltrates may affect
the deeper corium and the subcutaneous tissue in
some cases. Intravascular microthrombi and exten-
sive destruction of the vessel walls are not a feature
of Sweet's syndrome. In later stages, histiocytes and
lymphocytes accompanied by leukocytoc1asis pre-
dominate in the infiltrate. Jordaan subdivided an
early lymphocytic, an intermediate neutrophil-rich,
and a late histiocyticphase. He postulated that lym-
phocytes initially are present that subsequently ac-
tivate and attract neutrophils and histiocytes. Re-
current lesions, in our experience, contain fewer
neutrophils and are similar to late-stage lesions. In
our series, the formation of subcorneal pustules ac-
companied by a diffuse exocytosis of neutrophils
(Fig. 7) into the epidermis was demonstrable in 21%
of patients. In one patient we observed an initially
pustular variant that subsequently exhibited severe
blistering.i"
In contrast to other reports, Delabie et a1. 25found
no exocytosis of inflammatory cells in the epidermis,
whereas histiocytes, frequently with twisted pseu-
dosegmentednuclei,were the predominant infiltrat-
 Journal of the American Academy of Dermatology
540 von den Driescli October 1994

Fig. 5. Heterogeneous clinical lesions of Sweet's syndrome. A and B, Acneiform and

pseudopustular lesions in 29-year-old patient with Crohn's disease. C, Pustular lesions in 64-
year-old woman with Hashimoto's thyroiditis. D, Pustular and bullous lesions in 64-year-old
patient with idiopathic Sweet's syndrome. Note secondary pustules and blisters on erythe-
matous plaques . (D, From Tacke J, Diepgen TL, Albrecht HP, et al. Hautarzt 1994;45:185.)

ing cell type in the dermis. These cells may mimic
neutrophils and lead to an overestimation of the
total number of infiltrating neutrophils in a given
lesion. In Delabie's report, only two patients with
leukemia-associated Sweet's syndrome exhibited
neutrophil-dominated infiltrates.
The subcutaneous fat often contains moderate
perivascular infiltrates, especially in the septal ar-
eas.95 In EN-like lesions, a septal panniculitis with
Miescher's granulomas and some neutrophils is
characteristic.P- 66 A few reports describe a subtype
characterized by the occurrence of neutrophil-rich
infiltrates in the subcutaneous tissue.97 -99
INVOLVEMENT OF INTERNAL ORGANS
Lazarus et a1. 1OO first reported sterile lung involve-
ment characterized by an infiltration of mature
neutrophils. This neutrophilic alveolitis responds
rapidly to treatment with corticosteroids.!"' Lung
involvementhas often been reported in patients with
an underlying hematologic disorder.'02-105 Heart
involvement has been described in only one patient
Journal of the American Academy of Dermatology
Volume 31, Number 4 von den Driesch 541

Fig. 6. Other manifestations of Sweet's syndrome. A, Early pseudopustular and aphthoid

lesions affecting oral mucosa in Sweet's syndrome. B, EN-like lesions on lower leg. (A, From
von den Driesch P, von Hintzenstern J, Schlegel Gomez R, et al. Dermatol Bild 1990;
13:7.)

Fig. 7. Histologic findings in Sweet's syndrome. Bandlike infiltrate consists of perivascular

mononuclear cells and numerous neutrophils. A, Note exocytosis of cells through epidermis
to form small subcorneal pustules. B, Diffuse exocytosis of segmented-nuclear neutrophils
stained with ASD-granulocyte-esterase.

with lung infiltrates who suffered from pleuroperi-
cardiac effusionsimilar to Dressler's syndrome. 27, 106
Sterile osteomyelitis, responsive to therapy, is a rare
complication of Sweet's syndrome that has been ob-
served only in children. 38,40,41, 46
Severe renal involvement responsive to therapy
was first reported in a case of clinically simulating
systemic lupus erythematosus. 107 Two other patients
had acute renal failure and required dialysis. One
patient died of renal disease . 108 In one patient of our
series the level of serum creatinine rose from 1.0 to
5.0 mg/dl within 2 days, accompanied by a corre-
sponding decrease of creatinine clearance. During
therapy with methylprednisolone, serum creatinine
and creatinine clearance returned to normal within
9 days. Takahashi-P? described a patient with tran-
 Journal of the American Academy of Dermatology
542 von den Driesch October 1994

Table III. Laboratory values in 38 consecutive

patients with Sweet's syndrome
Laboratory Values %

ESR
-30 mm/hr 11
-90 mm/hr 60
>90 mrri/hr 29
Leukocytosis
<8,000 20
<10,000 20
<15,000 37
>15,000 23
Segmented-nuclear neutrophils + stabs
>70% 79
Shift to the left 50
(>7% stabs)
Lymphocytes <25% 76
Hemoglobin <13.0 gm/dl 54
/X2-globulins > 10% 90
C-reactive protein elevated 84
Alkaline phosphatase> 46
Fig. 8. Immunohistochemical finding in Sweet's syn- 103 IU/ml
drome: Double-staining of 46E5 (anti-IL-8 /NAP-1; gift ANCA (n = 10) 0
of Dr. M. Sticherling, Kiel, Germany), reactive spindleor ANeA, Anti-neutrophil cytoplasmic-(auto)antibodies; ESR, erythro-
dendritic cells with rhodamine isothiocyanate and factor cyte sedimentation rate.
VIII-reactive endothelial cells with fluorescein isothiocy-
anate. Note absence of any epidermal IL-8 immunoreac-
tivity.

sient involvement of kidney, liver, and pancreas.
Kemmett and Hunter reported slightly increased
levels of alkaline phosphatase in 83% and transient
elevations of hepatic AST (SGOT) and -v-glutamyl-
transferase (-y-GT) in 17%. We found moderate el-
evationsinhepaticASTin 4%, ofthe glutamate pyru-
vate transaminase (GPT) in 29%, of -y-GT in 39%,
and of alkaline phosphatase in 46%. After therapy,
45% (for -y-GT), 75% (for GPT), and 83% (for alka-
line phosphatase) of the enzyme elevations returned
to normal within 3 weeks, which suggests a direct
correlation to the underlying Sweet's syndrome.
There are three reports on neurologic involvement
in Sweet's syndrome. Aseptic meningitis was found
in a 7-week-old child, the youngest patient report-
ed. 48 Neurologic and psychiatric symptomsll" and
accumulation of neutrophils in the cerebrospinal
fluid III were present in other patients. These re-
sponded well to therapy.
LABORATORY FINDINGS
Table III summarizes the results of routine labo-
ratory studies in our 38 patients with Sweet's
syndrome. An elevated erythrocyte sedimentation
rate (ESR), segmented nuclear neutrophils and
stabs greater than 70% in blood smear examinations,
moderate lymphopenia, decreased hemoglobin, in-
creased a2-globulin fraction, and elevated serum
C-reactive protein levels were characteristic labora-
tory findings. Leukocytosis of more than 10,000/
mm', however, was present in only 60% of patients.
Recently, the presence of circulating autoantibodies
against neutrophilic cytoplasm antigens (ANCA)
was reported in five patients with Sweet's syn-
drome.P: 112 We could not find cytoplasmic or per-
inuclear ANCA in 10 consecutive patients. 113 This
indicates ANCA are not serologic markers for the
majority of the patients.
DIFFERENTIAL DIAGNOSIS (Table IV)
EM, in particular, may clinically closely resemble
Sweet's syndrome, which suggests a possible relation
between both diseases. 114 In a recent study of 32 pa-
tients with either EM or Sweet's syndrome, a com-
prehensive score was used. I IS The fact that 4 of 32
cases remained doubtful suggested an overlap. Our
group, in contrast, studied 45 cases of EM since 1985
and found no case to be indistinguishable from
Sweet's syndrome.
Disseminated forms of EN may clinically be dif-
ficult to separate but are histologically characterized
by infiltrates predominantly located in the septa of
 Journal of the American Academy of Dermatology
Volume 31, Number 4 von den Driesch 543

Table IV. Differential diagnosis of Sweet's syndrome

Skin lesions
Diagnosis Patients' history and symptoms Laboratory results Histologic features

Erythema Herpes simplex Target lesion with Parameters for Less neutrophils in all
multiforme infection; drug central blistering; inflammation types; hydropic
intolerance; no pustules; fever (ESR, basal cell
recurrence and arthralgia leukocytosis) degeneration in
less often normal or often epidermal type
only moderately
changed
Erythema nodosum Similar, including Confined to the Similar to Sweet's
Septal panniculitis in
associated lower legs;similar syndrome all lesions
diseases in disseminated
variants
Erythema elevatum Chronic disease Different Normal; in Vasculitis;
et diutinum with acute predilection sides; exacerbation immunofluorescence
exacerbations general malaise in phase similar positive*
exacerbation
Behcet's disease Chronic disease Genital aphthae, Standard Vasculitis;
with acute pustular skin parameters immunofluorescence
exacerbations lesions, similar; different positive*
thrombophlebitis, HLA pattern,
uveitis different increased
from Sweet's chemotaxis of
syndrome neutrophils 197
Chronic neutrophilic Chronic disease Skin lesions similar; No changes in ESR Indistinguishable
plaquesl98, 199 with recurrence no fever or and peripheral
general malaise leukocytes
Bowel-bypass Gastrointestinal Pustular lesions; Similar Vasculitis or similar to
syndrome surgery/disease fever and arthritis Sweet's syndrome
similar
Pyoderma Similar associated General malaise and Only in acute types Perivascular infiltrates
gangrenosum diseases; fever only in similar to Sweet's deeper; ulceration;
chronic disease acute types syndrome immunofluorescence
positive" in about
50%116

*Immunofluorescence: Direct immunofluorescence with antibodies against IgG , IgM, [gA, complement factors CIa, C3, and C4.

the subcutaneous tissue in all lesions. Since 1985 we
observed two patients with disseminated EN in
whom some lesions histologically resembled Sweet's
syndrome, which suggests some overlap between
both diseases. In addition, general symptoms and
standard laboratory findings are not useful for the
differentiation of both classic and disseminated EN
and Sweet's syndrome (manuscript in preparation).
Another patient with Crohn's disease first had
Sweet's syndrome with EN, 1 year later EN alone,
and 1 year later pyoderma gangrenosum alone.70, 116
The spectrum of associated conditions in EN (e.g.,
ulcerative colitis, Crohn's disease, Behcet's disease,
lymphomas, leukemias, sarcoidosis, pregnancy) is
also similar to Sweet's syndrome. Therefore we sug-
gest a close relation between both diseases and
include at least some variants of EN in the spectrum
of reactive neutrophilic dermatoses despite the fact
that histologically neutrophils are not predominant
in most lesions of EN.
Classic pyoderma gangrenosum is clinically easy
to separate, but acute disseminated vesiculopustular
forms in association with hemoproliferative disor-
ders or autoimmune bowel diseases as well as simul-
taneous occurrence of acute pyoderma gangre-
nosum and Sweet's syndrome may be difficult to
distinguish. 10. 57,82. 117-128
Bowel bypass syndrome occurs after jejunoileal
bypass surgery, the Occurrence of a blind loop after
peptic ulcer surgery, or in association with inflam-
matory bowel disease. 129,130 Papulopustular lesions
as well as fever and arthralgias similar to the find-
ings in Sweet's syndrome occur. The histopathologic
changes may be identical and have been termed

544 von den Driescb
Table V. Revised version of the diagnostic
criteria initiated by Su and Liu for the diagnosis
of Sweet's syndrome"
Major criteria
1. Abrupt onset of tender or painful erythematous
plaques or nodules occasionally with vesicles,
pustules or bullae
2. Predominantly neutrophilic infiltration in the
dermis without leukocytoclastic vasculitis
Minor criteria
1. Preceded by a nonspecific respiratory or
gastrointestinal tract infection or vaccination or
associated with:
• Inflammatory diseases such as chronic
autoimmune disorders, infections
• Hemoproliferative disorders or solid
malignant tumors
• Pregnancy
2. Accompanied by periods of general malaise and
fever (>38° C)
3. Laboratory values during onset: ESR >20 mm;
C-reactive protein positive; segmented-nuclear
neutrophils and stabs >70% in peripheral blood
smear; leukocytosis >8000 (three of four of these
values necessary)
4. Excellent response to treatment with systemic
corticosteroids or potassium iodide
"'Both major and two minor criteria are needed for diagnosis.
neutrophilic vascular reaction or Sweet's-like vas-
culitis.!3! The borderline between both diseases has
been questioned and they may be close clinical vari-
ations of the same pathogenic process. 82, !22
Behcet's disease may be clinically similar to
Sweet's syndrome. Characteristic features of Beh-
~t's disease (e.g., genital aphthae, recurrent uveitis,
superficial thrombophlebitis, involvement of the
central nervous system, a family history of the dis-
ease, chronicity, and a distinct HLA pattern) pro-
vide criteria for differentiation. 132
Neutrophilic eccrine hidradenitis is associated
with hemoproliferative diseases under treatment
with chemotherapeutic agents and may cause
Sweet's syndrome-like lesions and symptoms ac-
companied by drug-induced neutropenia.P'' Histo-
logically, differentiation to Sweet's syndrome is pos-
sible because the neutrophilic infiltrates are located
around or within eccrine sweat glands. !34
Symmetrically distributed lesions on the face oc-
casionally occurring in Sweet's syndrome may be
similar to lupus erythematosus.54, 107 The latter can
be easily excluded by histologic findings. In hydrala-
zine-induced subacute-cutaneous lupus erythema-
tosus, Sweet's syndrome is not rare.135-138
Journal of the American Academy of Dermatology
October 1994
Toxic pustuloderma is a severe hypersensitivity
reaction to drugs such as carbamazepine.P'" 140 Fe-
ver, leukocytosis, and involvement of lymph nodes,
liver, and kidney may occur. The histologic findings
may be similar to pustular Sweet's syndrome, but
the finding of disseminated, predominantly solitary
pustules with and without binding to hair follicles
allows the differentiation.
Other conditions to be distinguished from Sweet's
syndrome are erythema elevaturn diutinum (Table
IV), leukocytoclastic vasculitis, periarteritis nodosa,
halogenodermas, chloroma, granuloma faciale, and
infectious disorders such as impetigo contagiosum,
erysipelas, and bacterial sepsis.l'"
Histologically, early lesions of granuloma faciale,
erythema elevatum diutinum, bullous pyoderma
gangrenosum, Behcet's disease, bowel bypass syn-
drome, pustular psoriasis and sub corneal pustular
dermatosis, acute dermatitis with superinfection,
secondarysyphilis, neutrophilic arthropod reactions,
erythropoietic protoporphyria, and familial Medi-
terranean fever may be similar (outlined in Jor-
daan'"), and a correlation between clinical and his-
topathologic findings is necessary for diagnosis.
DIAGNOSTIC CRITERIA
Su and Liu8 proposed two major and four minor
criteria for the diagnosis of Sweet's syndrome that
have been widely accepted. Whereas their major
features are not contested, we use a revised version
of the minor criteria (Table V). All of our patients
fulfilled at least two of the new minor criteria.
PATHOGENESIS
It is generally accepted "that the condition is a
reaction to something or other . . . " as stated by
Sweet in his original report. However, two main
questions remain to be answered: Why do some pa-
tients with Sweet's syndrome react, and how is this
reaction established? The predisposition of women
toward classic and parainflammatory forms of
Sweet's syndrome and related disorders such as
EN,?! pyoderma gangrenosum.U'' and subcorneal
pustulosis of Sneddon-Wilkinsoa'V suggests that
the hormone system may influence the incidence and
pathogenesis of acute or chronic neutrophilic reac-
tions. Correspondingly, association of Sweet's syn-
drome with the administration of an oral contracep-
tive was reported.F'
Neutrophilic dermatoses may be associated with
distinct HLA subtypes. Mizoguchi et al.56 reported
a significantly increased occurrence of HLA-Bw54
Journal of the American Academyof Dermatology
Volume 31, Number 4
Table VI. Inflammatory diseases associated with Sweet's syndrome
Associated condition
Behcet's disease
Crohn's disease
Ulcerative colitis
Sjogren's syndrome
Lupus erythematosus
Thyroiditis
Rheumatoid arthritis
Mixed connective tissue disease
Yersiniosis
Typhus
Sepsis
Salmonellosis
Toxoplasmosis, histoplasmosis,
ureaplasmosis
Mycobacteriosis (in hairy cell leukemia)
Tuberculosis
Cytomegalovirus infection
Lepra
Focal infections (tonsillitis,
vulvovaginal infections)
Chronic active hepatitis
HIV infection
(relative risk 5.1). Our study of 38 Caucasian pa-
tients compared with 162 healthy controls revealed
only a not significantly increased frequency of
HLA-B8 and HLA-Cw7 (relative risk about 2).
Activation and mobilization of neutrophils and
histiocytes in Sweet's syndrome suggest the presence
of local deposits of immunoglobulin complexes and
complement on vessel walls as in type III hypersen-
sitivity. Immunohistochemical studies on single or
few cases indeed demonstrated deposits of immuno-
globulins or complement in the lesions.39, 64, 144-147
However, Going et a1.l48 and our group (unpub-
lished observations) found no deposition of immu-
noglobulins in 35 patients studied. Only in older le-
sions were discrete and probably secondary deposits
of complement factor C3 on vessel walls revealed.
Analysis with the Clq-binding assay of eight sera of
our patients Sweet's syndrome showed circulating
immune complexes in only one case (unpublished
observations). These findings suggest that other or
additional pathogenic processes are relevant in
Sweet's syndrome.
Other findings suggest an antigen- or superanti-
gen-induced, T'-cell-dependent cellular immune
reaction underlying the disease. The presence
of CD25+ activated T cells in the inflammatory
infiltrates has been demonstrated.e- 149, 150 A
recent immunohistochemical analysis of adhesion
von den Driescb 545
Reference Nos.
56, 62, 200-202
15,22,27,70,203,204
I, 15, 22, 23, 27, 125, 204-206, this report
207,208, this report
135-138
209,210, this report
22,24,86,211,212, this report
213
214-216, this report
217
218
219
192,220, 221
222
191,223
84
224
3,8,27,28,57,63, 76, 191,225
226
227
molecule expression in eight consecutive cases
demonstrated prominent intercellular adhesion
molecule-l expression on lesional keratinocytes in
four cases.150 This suggests activation by interferon
gamma released by T lymphocytes. A cytokine
cascade may explain the local and systemic activa-
tion of neutrophils and histiocytes. Release of inter-
leukin 1, interleukin 3, interleukin 6, interleukin
8, granulocyte colony-stimulating factor (G-CSF),
granulocyte-monocyte CSF (GM-CSF), and inter-
feron gamma may account for several characteris-
tic clinical symptoms and immunopathologic find-
ings observed in Sweet's syndrome. 18, 151,152 Using
specific monoclonal antibodies for interleukin 8,153
a cytokine with strong chemotactic properties for
neutrophils and lymphocytes, we found interleukin
8-reactive dendritic cells in the dermis of 4 of 10
patients (Fig. 8), whereas in EM (n = 5) and normal
skin (n = 10) no reactivity was revealed. Similar
to the findings in psoriasis.P" the epidermal inter-
leukin 8 immunoreactivity was diminished in all
cases. However, the serum levels of interleukin
8 as measured by enzyme-linked immunosor-
bent assay, were normal in these patients (Sticher-
ling M, von den Driesch P, unpublished observa-
tions).
The role of hemopoietic mediators in pathogene-
sis is supported by the fact that Sweet's syndrome

546 von den Driesch
Table VII. Hemoproliferative diseases associated with Sweet's syndrome
Associated hemoproliferative disease
Myelodysplasiajmyelofibrosis
Myeloid proliferation j myeloid
metaplasiajpreleukemia
Acute leukemia (myelogenous or
unclassified)
Acute myelomonocytic leukemia
Acute megakaryoblastic leukemia
Acute lymphoblastic leukemia
Chronic myeloid leukemia
Multiple myeloma
Hairy cell leukemia
Chronic lymphocytic leukemia
Chronic neutrophilic leukemia
Different forms of anemia
Gammopathy
Different forms of polycythemia
Hodgkin's lymphoma
Non-Hodgkin's lymphoma
may develop during G-CSF therapy for granulope-
nia.155-160 In one patient with leukemia-associated
Sweet's syndrome, increased levels of G-CSF and
interleukin 6 in serum were demonstrated.l''! Ex-
cessive production and elevated serum levels of
G-CSF and GM-CSF have been found with diseases
associated with neutrophilic dermatoses such as in-
fections, myelodysplastic syndromes, leukemias, and
solid carcinomas.162.167
Other investigators suggest altered functions of
neutrophils in the pathogenesis of Sweet's syndrome.
Abnormal lysosomal enzyme activityl68 and a de-
crease of "oxidative burst,,169 have been reported.
Analyses of chemotactic properties of neutrophils in
Sweet's syndrome revealed. contradictory results.
Either increased, decreased, or normal chemotaxis
has been found. 127, 145. 169-174 We observed decreased
chemotactic migration of granulocytes isolated dur-
ing the acute phase of seven consecutive patients
with Sweet's syndromel" and interpreted these
findings as probably an epiphenomenon. Our
chemotaxis assays with the sera of these pa-
tients and granulocytes of healthy donors failed to
confirm the results of Kaplan et al.,176 who found a
heat-stable chemotactic factor in the plasma of one
patient.
In summary, none of these three principal con-
cepts (e.g., immunocomplex vasculitis, T-cell acti-
vation, altered functions of neutrophils) is definitely
supported by experimental results.
Journal of the American Academy of Dermatology
October 1994
Reference Nos.
45, 51,99, 102, 104, 123, 156, 228-249
10, 11, 102,250-254
6-8,11 ,22,25,27,55,76,99,102,118, 177, 183,
239, 251, 253, 255-282
27,97,272,283
284
44
24, 27,42, 51, 103, 193, 211, 275, 285-292
28, 123, 293-296
51, 155,222,297-300
301-305
306
45,46, 127, 157,307-309
22, 244, 310
23,124,253,311-315
15,51,259
22,23,278,316-323, this report
ASSOCIATED DISEASES
Tables VI-IX summarize the world literature
about diseases associated with Sweet's syndrome.
The major parainflammatory diseases are au-
toimmune or infectious (Table VI). Sweet's syn-
drome associated with hemoproliferative diseases
(Table VII) may precede the underlying disorder for
years-' and in general worsens the prognosis.l'' An
unusual early onset of side effects from therapy in
patients with leukemia has also been reported.!"?
The rising number of cases found in association
with solid carcinomas (Table VIII) supports the
concept that these paraneoplasias did not occur by
chance. In contrast to the majority of other parane-
oplastic diseases, Sweet's syndrome can occur so
early in the development of these cancers that cur-
ative therapy is possible.
No fundamental clinical and histopathologic dif-
ferences between classic and paraneoplastic Sweet's
syndrome are known. As pointed out by Cohen et
a1., 18 the female/male ratio in paraneoplastic cases
is about 1:1; thus male patients have a higher risk of
being affected by paraneoplastic Sweet's syndrome.
The features of no antecedent respiratory tract
infection, heterogeneity of cutaneous lesions with
blistering and/or ulceration, recurrence of Sweet's
syndrome, and abnormal laboratory results (e.g.,
anemia, thrombocytosis, thrombocytopenia) require
thorough diagnostic investigations.19, 20 Frequent
observations of these patients is also advisable.
Journal of the American Academy of Dermatology
Volume 31, Number 4 von den Driesch 547

Table VllI. Solid carcinomas associated with Sweet's syndrome

Associated cancer of the: Reference Nos.

Breast 15, 237, 304, 305, 324-327

Postmastectomy syndrome 328
Stomach 22, 329, 330
Prostate 245, 331, 332, this report
Uterus and vagina 15,329,333,334
Colon and rectum 196,335-337
Testis 5
Lung and bronchus 24,338
Larynx 20
Thyroid gland 339
Bladder 25
Triple (oat cell, squamous cell of 340
the lung and prostate)
Kidney 341
Melanoma 197
Unknown 20,244

Table IX. Miscellaneous conditions associated with Sweet's syndrome

Associated condition Reference Nos.

Pregnancy 11,22,63,191,342-344, this report

Vaccination 21,131,345,346
Complement deficiency 347
Subacute necrotizing lymphadenitis 348
Sarcoidosis 72,349
Ureter obstruction 350
POEMS syndrome 351
Exogenous contact with pepper 352
POEMS, Polyneuropathy,organomegaly, endocrinopathy, M protein,skin changes.

Sweet's syndrome is only rarely associated with
drug sensitivity. MinocycIine,I78-180 lithium.P! and
furosemide-P have been reported as possible agents.
More recently, development duringthe treatment of
leukemia with the differentiation-inducing drug all-
trans-retinoic acid has been reported.l83, 184
THERAPY AND FOLLOW-UP
The standard therapy for Sweet's syndrome is
prednisone or prednisolone at an initial dose of 0.5
to 1.5 rug/kg of body weight per day. Gradual
reduction is recommendedwithin 2 to 4 weeks. This
therapy leads to a remission of the general malaise
within hours; of mucosal lesions, fever, and arthral-
gias within 2 days; and of skin lesions within 7 (3 to
9) days. Abnormal laboratory findings also rapidly
return to normal.l'"
Despite this good initial responseto therapy, the
disease is characterized by frequent recurrence.
Kemmett et aI.22 reported that 21% oftheir patients
had more than oneepisodeof Sweet's syndrome and
another 10% had chronic relapsing disease for at
least 3 years. In Spain, 30% of the patients had two
to six episodes of Sweet's syndrome.P Follow-up of
our patients revealed short-term relapses after the
end of the therapy in 25 %, occurrence ofthe chronic
relapsing form in 15%, and recurrence after a free
interval of more than 12 months in another 8%.
Alternatives to corticosteroid treatment include
potassium iodide, colchicine, dapsone, doxycycline,
clofazimine, nonsteroidal antiinflammatory agents
and cyclosporine (Table X). An influenceon migra-
tion and other functions of neutrophils is the com-
mon linkbetweenthese drugs. Potassiumiodide is as
effective as corticosteroids, 186,187 and the risk of
short-term relapses may be Iower.l'" However, in
two patients treated with potassium iodide a severe
vasculitis developed that required high doses of ste-

548 von den Driesch
Table X. Nonsteroidal treatment modalities for Sweet's syndrome
Recommended initial
Drug dose (mg/day)
Potassium iodide 900
Colchicine 1.5
Dapsone 100-200
Clofazimine 200
Cyclosporine 5-10*
Indomethacin 50-100
Naproxen 750
Doxycycline 200
"Per kilogram of body weight.
roids for therapy.F: 188 Koranda'j? and subsequent
investigatorsl-v 172 reported dapsone to be effective
in Sweet's syndrome. Two patients, however, who
had been unsuccessfully treated with dapsone re-
sponded to potassium iodide.173 Tetracycline and
doxycycline also have been used successfully in pa-
tients .190, 191
Other reported treatments include nonsteroidal
antiinflammatory agents such as indomethacin15,192
or naproxen, 193 chlorambucil and methylprednisolo-
ne,194 or cyclophosphamide.l'" Long-lasting post-
lesional erythema, not infrequent in Sweet's syn-
drome, may respond to UVB phototherapy.l'"
In summary, oral administration of corticoster-
oids represents the "standard" of treatment. Potas-
sium iodide is a widely used alternative but may
cause severe vasculitis. Administration of dapsone,
colchicine, c1ofazimine, cyclosporine, and other
drugs should also be considered as alternatives and
used primarily in patients with recurrent Sweet's
syndrome.
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