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Background

This interesting skin rash was first described by Robert Douglas Sweet in 1964 as Acute Febrile Neutrophilic Dermatosis. Ever since his description, the disease has been referred to his name. As it was first described, the classic symptoms consist of an acute onset of erythematous plaques, nodules, and occasionally pustules, assymetrically distributed over the face, neck, and extremities. This eruption is often accompanied with fever and a neutrophilic leukocytosis. The rash may last from 1 week to several years.

The importance of correctly identifying this disease lies in its association with other diseases. An overview of some of these diseases is presented in the outline below. Some investigators have preferred the term Neutrophilic dermatosis of myeloproliferative disorders when this disease is associated with myeloproliferative disorders.

EPIDEMIOLOGY CHARACTERIZATION
SYNONYMS Acute febrile neutrophilic dermatosis
Sweet's disease
AGE RANGE-MEDIAN Mid to late 50's
SEX (M:F)
Females

 

DISEASE ASSOCIATIONS CHARACTERIZATION
Malignancy or hematopoietic disease

11-54%

Hematologic malignancy most common with acute myelogenous leukemia and myelodysplasia the most frequent
Wide variety of solid tumors
No female predominance
More often pustular/bullous
More localized to face/upper body

Immunologic disease

19%

Relapsing polychondritis
Rheumatoid Arthritis
Dermatomyositis
Non-specific connective disease
Inflammatory Bowel Disease

Infections

URI
UTI
V iral pneumonia
Yersina infection
Typhus
Salmonellosis
Toxoplasmosis
Tonsillitis
Hepatitis
Vulvovaginitis
Helicobacter pylori infection

Sweet's syndrome and Chlamydia pneumoniae infection

Pietro Rubegni, etal.

J Am Acad Dermatol 2001;44:862-4 Abstract quote

We report the case of a patient in whom Sweet's syndrome developed during pneumonia caused by Chlamydia pneumoniae. Increased expression of helper T-cell type 1 cytokine secretion pattern in peripheral blood has recently been observed in patients with this syndrome, and chlamydia infection is known to primarily activate a helper T-cell type 1 immunologic response.

HERPES SIMPLEX
 


A case of Sweet's syndrome developed after the treatment of herpes simplex infection in a metastatic breast cancer patient.

Coskun U, Gunel N, Senol E, Ilter N, Dursun A, Tuzun D

J Cutan Pathol 2002 May;29(5):301-4 Abstract quote

Background: Sweet's syndrome or acute febrile neutrophilic dermatosis is associated with several systemic diseases such as malignancies and infectious diseases.

Methods: We present a 34-year-old woman with Sweet's syndrome associated with both herpes infection and metastatic disease.

Results: Skin biopsy showed neutrophilic infiltrates in the dermis confirming the diagnosis of Sweet's syndrome.

Conclusions: To our knowledge, this is the second case of Sweet's syndrome associated with herpes simplex infection in the literature. Further observations are required to determine the relationship between Sweet's syndrome and herpetic infection.

Drug related All trans-retinoic acid
TMP-sulfamethoxazole
H ydralazine
Oral contraceptives
Minocycline
Li
Furosemide

Celecoxib-induced Sweet's syndrome

Kenneth H. Fye, etal.

San Francisco, California

J Am Acad Dermatol 2001;45:300-2. Abstract quote

Sweet's syndrome and related neutrophilic dermatoses have been associated with a variety of medications. Celecoxib is a new cyclo-oxygenase-2 inhibitor recently approved for arthritis.

We describe a 57-year-old man who experienced tender pustulopapular lesions on the dorsal aspects of the hands, neck, and legs 1 week after starting celecoxib.

Histopathologic examination of the lesion showed a diffuse dermal neutrophilic infiltrate, edema of the papillary dermis, spongiform pustules, and no leukocytoclastic vasculitis. These findings were consistent with Sweet's syndrome. Without realizing a possible association, the patient rechallenged himself with a second course of the medication, which resulted in a rapid exacerbation of his lesions. After discontinuing the medication for the second time, the patient has had complete clearing of his lesions.

To our knowledge, this is the first report of Sweet's syndrome associated with this new class of nonsteroidal anti-inflammatory drugs.

Pregnancy related 2%
All presented 1st or 2nd trimester
Most resolved spontaneously
No fetal morbidity or mortality

 

PATHOGENESIS CHARACTERIZATION
Possible hypersensitivity reaciton Type of hypersensitivity reaction which leads to stimulation of a cascade of cytokines that precipitate neutrophil activation and infiltration
A T-cell mediated immune response has been postulated
HLA J Am Acad Dermatol 1987;37:276-278.
No consistent association
Clonal neutrophilic dermatosis in the setting of CD34 positive AML treated with granulocyte colony stimulating factor

J Cutan Pathol 2001;28:90-96

One case
May reflect therapy induced differentiation of sequestered leukemic cells
Neutrophils displayed dysplastic features

 

LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION
Laboratory Markers  
ESR >90% of cases
Leukocytosis >8000 in 80% of cases
Less common in drug associated cases
Anemia 54%
Usually associated with malignancy
Low platelets Usually associated with malignancy
Alkaline phosphatase elevation 46% of patients
ANCA positive J Am Acad Dermatol 1994;31(4)535-556.
None

 

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION
General  
VARIANTS  
Oral mucosa  
Arthralgia/arthritis (33-62%)  
Lungs(alveolitis)  
Kidney  
Liver  
Bone(sterile osteomyelitis)  
Central Nervous System  
Neutrophilic Dermatosis of the Dorsal Hands
(Pustular vasculitis of the hands)

J Am Acad Dermatol 2000;43:870-4
Patients with this condition presented with low-grade fevers and erythematous plaques, pustules, and bullae limited to the dorsal hands and fingers

Biopsy specimens showed a neutrophilic infiltrate and leukocytoclasis, but no necrotizing vasculitis, and were reminiscent of Sweet's neutrophilic dermatoses

Corticosteroids or dapsone led to clearing of the lesions, and small maintenance doses of dapsone prevented their recurrence
Low-dose dapsone is proposed as a possible first-line therapy in this condition, especially in those with recurrent disease

Neutrophilic myositis as an extracutaneous manifestation

J Am Acad Dermatol 2001;44:137-139

Initial presentation in patient who later presented with pyoderma gangrenosum

Sweet's syndrome in acute myelogenous leukemia presenting as periorbital cellulitis with an infiltrate of leukemic cells

Kelli W. Morgan, MD
Jeffrey P. Callen, MD

Louisville, Kentucky

J Am Acad Dermatol 2001;45:590-5 Abstract quote

Sweet's syndrome is characterized by the abrupt onset of fever, neutrophilic leukocytosis, and erythematous, tender pseudovesiculated plaques or nodules that respond readily to corticosteroid therapy. It is usually distinguished by the presence of mature neutrophils on histopathologic examination.

We describe a 38-year-old man with acute myelogenous leukemia who had an erythematous vesicular eruption of the left eye develop that resembled cellulitis.

A biopsy specimen revealed a dermal infiltrate of mature neutrophils and immature myeloblastic precursors. He later had hemorrhagic pseudovesiculated plaques develop bilaterally on his hands. A biopsy specimen again revealed abundant neutrophils with immature forms. A similar eruption developed at the site of a Hickman catheter placement 4 months later. His skin lesions responded rapidly to oral corticosteroids.

This case is unique in that his initial presentation of Sweet's syndrome resembled orbital cellulitis that was characterized by immature myeloid precursors on histopathology.

 

HISTOLOGICAL TYPES CHARACTERIZATION
General

Dense nodular or diffuse dermal infilrate of neutrophils, nuclear dust, dermal edema, with no evidence of vasculitis

Focal involvement of the subcutaneous fat may occur

VARIANTS  
IMMATURE MYELOID CELLS  
Immature myeloid precursors associated with myelodysplastic syndrome Am J Dermatopathol 2000;22:429-433

Atypical cells had identical phenotype to the leukemic cells in the peripheral blood and bone marrow
VASCULITIS  
Vascular Inflammation (Vasculitis) in Sweet Syndrome

A Clinicopathologic Study of 28 Biopsy Specimens From 21 Patients

Janine C. Malone, MD; Stephen P. Slone, MD; Lisa A. Wills-Frank, MD; Paul K. Fearneyhough, MD; Sheron C. Lear, HT(ASCP), HTL, QIHC; L. Jane Goldsmith, PhD; Antoinette F. Hood, MD; Jeffrey P. Callen, MD

 

Arch Dermatol. 2002;138:345-349 Abstract quote

Background
Sweet syndrome is characterized by painful, erythematous plaques of rapid onset accompanied by fever. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. We hypothesized that vasculitis can occur in Sweet syndrome and that it represents an epiphenomenon rather than a primary immune-mediated process.

Design
Skin biopsy specimens from patients with Sweet syndrome were reviewed to determine the prevalence of vasculitis. The clinicopathologic features of cases with vasculitis were evaluated for statistically significant associations. Specimens with vasculitis underwent immunofluorescence staining.

Setting
University department of dermatology, university hospital, and private practice.

Patients
Medical records and biopsy specimens of 21 patients meeting diagnostic criteria for Sweet syndrome were reviewed.

Interventions
None.

Results
The prevalence of vasculitis was 29% (6 of 21 patients). There was a significant association of vasculitis with lesions of longer duration (P = .02). Vascular immunoglobulin and complement could not be demonstrated in cases of Sweet syndrome with vasculitis.

Conclusions
Vasculitis is not a primary, immune-mediated process in Sweet syndrome but occurs secondary to noxious products released from neutrophils. Blood vessels in lesions of longer duration are more likely to develop vasculitis than those of shorter duration because of prolonged exposure to noxious metabolites. Vasculitis does not exclude a diagnosis of Sweet syndrome.

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
Bowel bypass-related dermatosis  
Cellulitis/erysipelas  
Disseminated erythema nodosum  
Erythema elevatum diutinum  
Leukocytoclastic vasculitis  
Pustular vasculitis of the dorsal hands  
Pyoderma gangrenosum  

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
Recurrence Even after systemic steroid treatment, 25-37% of cases may recur
Treatment Corticosteroids
Systemic corticosteroids
NSAIDs
Experimental Therapies  
Colchicine
 
Clofazimine
 
Pentoxifylline
 
Dapsone
 
Doxycycline
 
Cyclosporine
 
Potassium iodide
 

J Am Acad Dermatol 1983;9:751-758
Am J Dermatopathol 1989;11:99-111
J Clin Oncol 1988;6:1887-1897.


Commonly Used Terms

Myelodysplastic Syndrome

Rashes


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Last Updated 8/6/2002

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The Doctor's Doctor