AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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INTRODUCTION

Section 2 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
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• Medication
• Follow-up
• Miscellaneous
• Multimedia
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Background

Acute febrile neutrophilic dermatosis, also termed Sweet syndrome, is a reactive process characterized by the abrupt onset of tender, red-to-purple papules, and nodules that coalesce to form plaques. The plaques usually occur on the upper extremities, face, or neck and are typically accompanied by fever and peripheral neutrophilia.

Initially described in 1964 by Robert Sweet, the entity currently recognized as Sweet syndrome ranges from classic Sweet disease, which occurs in young women after a mild respiratory illness, to a more aggressive neutrophilic process, which may be associated with other inflammatory diseases or malignancy. In fact, the lesions may be the first evidence of an underlying disorder and should prompt further investigation. A drug-induced variant due to the administration of various medications has been recognized, and a pregnancy-associated form has also been reported.

In general, Sweet syndrome responds dramatically to oral corticosteroids and may improve or resolve with treatment of the underlying condition. Without treatment, the syndrome may persist for weeks or months and then improves without leaving scars. Recurrences are common. In rare cases, crops of lesions reappear and the condition persists indefinitely. Cases associated with malignancy can be bullous or ulcerative and resemble atypical pyoderma gangrenosum. These lesions are often recalcitrant to treatment.

The diagnosis of Sweet syndrome is based on both clinical and histopathologic findings. Characteristics that distinguish the lesions of Sweet syndrome from other neutrophilic dermatosis are healing of the lesions without scarring and an absence of vasculitis.

Pathophysiology

Acute febrile neutrophilic dermatosis is a reactive process (a hypersensitivity reaction) that occurs in response to systemic factors, such as hematologic disease, infection, inflammation, vaccination, or drug exposure. The condition is neutrophil mediated, as evidenced by its histopathologic appearance, associated neutrophilia, and response to medications that affect neutrophil activity.

The association of exogenous granulocyte colony-stimulating factors (G-CSF) with the development of Sweet syndrome also supports the importance of neutrophils and related endogenous cytokines in the underlying process. G-CSF suppresses apoptosis and prolongs the survival of neutrophils in vivo in a CD34⁺ cell population. G-CSF levels in peripheral blood are increased in patients with active Sweet syndrome, suggesting that high levels of G-CSF may one day be a useful indicator of activity level of the disease. It is the functional properties of neutrophils rather than the absolute number that is thought to be significant, as patients with Sweet syndrome due to G-CSF develop lesions as the neutrophil count is rapidly increasing, despite the absolute neutrophil count being low.

In addition, some studies have suggested a role for tumor necrosis factor, and others have suggested an imbalance of type 1 helper T cells. Although the skin is the primary organ affected, other systems, most notably the lungs and kidneys, can also be involved. Other reactive neutrophilic disorders, such as neutrophilic eccrine hidradenitis, are closely related and may represent a spectrum with related pathogenesis.

A possible genetic link with HLA-B54 has been observed in the Japanese population. A report of 2 brothers who developed Sweet syndrome in the neonatal period also supports a genetic predisposition. Structural alterations in the long arm of chromosome 3 (3q) have been seen in association with Sweet syndrome; these changes involve genes that affect the regulation of granulopoiesis and neutrophil migration.

Frequency

International

Sweet syndrome is uncommon but not rare. Several hundred cases have been reported in the literature. In several series, 15-20% of cases have occurred in a setting of malignancy, though most are idiopathic or associated with benign conditions.

Mortality/Morbidity

Most cases of acute neutrophilic dermatosis resolve, though some persist indefinitely and can be difficult to manage because of pain and skin breakdown. Because this condition can be associated with many other diseases, including malignancy, the patient's overall prognosis depends on the underlying cause.

Race

Sweet syndrome has no known racial predilection.

Sex

The common reactive variant of Sweet syndrome does have a female predominance, with a female-to-male ratio of 2-3:1. However, this predilection was not noted in series of cases associated with malignancy.

Age

• Typically, women with Sweet syndrome are aged 30-50 years.
• Cases in neonates as young as 10 days have been described.
• In children, Sweet syndrome is extremely rare and generally associated with infection.

CLINICAL

Section 3 of 11  

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History

• Fever typically precedes the appearance of each crop of lesions. The fever can precede the skin disease by several days to weeks; however, it may also occur simultaneously.
• The crop of plaques or nodules often appears abruptly and may persist for days to weeks.
• Many patients report a febrile upper respiratory tract infection, tonsillitis, or flulike syndrome 1-3 weeks prior to onset of skin lesions. Vaccination or a gastrointestinal tract infection may also precede the eruption.
• Headache, malaise, and arthralgias are common.
• Episodes of disease cluster in the spring and autumn.

Physical

• Skin manifestations
• • Typical skin lesions are reddish blue or violet papules, plaques, or nodules. Massive subepidermal edema sometimes produces a deceptively vesicular appearance. Lesions may be studded with pustules. Papules often coalesce into circinate or arcuate plaques 2-10 cm in diameter.
  • Plaques can cause pain and burning, but they are not pruritic. Lesions spontaneously resolve without scarring, or they resolve after treatment.
  • The face, neck, and extremities primarily are affected, characteristically in an asymmetric distribution.
  • Atypical presentations in the external auditory canal and tympanic membrane are reported. Facial cellulitis and soft tissue infections of the extremities have also been described.
  • Ulcers and bullae are more common in malignancy-associated disease than in other forms. These lesions may be extensive and are generally hard to treat.
  • Lesions on the dorsum of the hand are not uncommon. They sometimes appear vasculitic, which is not typically seen in Sweet syndrome.
    • The lesions are predominantly distributed over the dorsal aspects of the fingers and hands in a roughly symmetrical pattern. Other extensor surfaces may also be involved.
    • Some believe that this is an anatomically limited form of Sweet syndrome, whereas others categorize this as a primary vasculitis.
    • Atypical pyoderma gangrenosum, bullous Sweet syndrome, and pustular vasculitis of the hands are actually considered by some to be variations of a single disease, neutrophilic dermatosis of the dorsal hands (Walling, 2006).
    • The vasculitis does not appear to be a primary immune-mediated process, as seen in the primary leukocytoclastic vasculitides, but rather, it is secondary vascular damage caused by toxic metabolites and proteases released from the extensive acute neutrophilic infiltrates in the skin. Prolonged exposure may increase the extent of damage.
• Mucosal lesions
• • Oral lesions can occur on the lips, buccal mucosa, and/or tongue. These lesions most commonly appear as ulcers in Sweet syndrome patients with hematologic disorders.
  • Conjunctivitis and episcleritis may also occur; these are the most common eye manifestations. Other ocular manifestations reported include uveitis, limbal nodules, glaucoma, subconjunctival hemorrhage, scleritis, and iritis (Levy, 2005).
• Extracutaneous manifestations
• • Sweet syndrome can involve several organ systems.
  • Pulmonary manifestations can sometimes lead to substantial morbidity. Pulmonary involvement may manifest as dyspnea, chronic cough, or pulmonary infiltrates or effusions on chest radiograph. In rare cases, symptoms may become severe enough to cause respiratory failure or bronchiolitis obliterans organizing pneumonia. Fortunately, most cases of Sweet syndrome with pulmonary involvement tend to be highly responsive to glucocorticoid therapy (Astudillo, 2006).
  • Other extracutaneous sites that have been reported include the bones, intestines, joints, bone marrow, pancreas, liver, heart, muscles, spleen, and kidneys.
  • Fewer than 30 cases of CNS involvement are reported in the literature. Encephalitis and meningitis are common neurologic manifestations in these cases. Peripheral neuropathy has also been reported. The most common symptoms are headaches, disturbed consciousness, and seizures (Hisanaga, 2005).
    • HLA types B54 and CW1 are associated with Sweet syndrome with CNS involvement in Japanese patients.
    • Unlike Behçet syndrome, in which CNS involvement is progressive and severe, Sweet syndrome usually causes transient CNS involvement, but recurrences may occur.
  • Proteinuria, hematuria, and decreased creatinine clearance have been reported.
  • Cerebrospinal fluid pleocytosis also has been described, as has a sterile chronic recurrent multifocal osteomyelitis in children.
• Pathergy
• • Like pyoderma gangrenosum, Sweet syndrome is known to cause pathergy (also referred to as Köebner phenomenon), in which lesions occur in areas of minor trauma, such as sites of scratches, bites, and venipuncture.
  • The lesions may also be photodistributed or localized to the site of a previous phototoxic reaction (eg, sunburn).

Causes

Potential causes are numerous, but some associations are well documented. Classic Sweet syndrome is the most common presentation and accounts for more than 50% of cases. Sweet syndrome associated with a (malignant) neoplasm accounts for approximately 20-25% of the cases. Most of these are hematopoietic malignancies (most commonly acute myeloid leukemia), but 15% are due to solid tumors, mostly those involving the genitourinary, breast, and gastrointestinal tract. Inflammatory (infectious) conditions are the next most frequently identified causes of Sweet syndrome.

• Hematologic malignancy
• • Myelodysplasia and chronic myelogenous leukemia may be associated with Sweet syndrome. Sweet syndrome can also be seen in association with acute myeloid leukemia (AML), including the promyelocytic (M3) variant of AML.
  • Other nonmyeloid hematologic malignancies that have occurred in association with acute febrile neutrophilic dermatosis include Hodgkin disease, cutaneous T-cell lymphoma, non-Hodgkin lymphoma, hairy cell leukemia, and multiple myeloma. (Patients with immunoglobulin G [IgG] secretion may be at increased risk for Sweet syndrome).
  • Nonhematologic malignancy has been associated with Sweet syndrome; rates of genitourinary, breast, and gastrointestinal cancers appear to be slightly increased in this group.
• Nonhematologic malignancy: Other rarely reported associations include osteosarcoma, oral cancer/tonsil cancer, ovarian cancer, thyroid cancer, lung cancer, pheochromocytoma, and rectal carcinoma.
• Multiple infections are described in association with Sweet syndrome.
• • These infections often involve the upper respiratory tract. Streptococcal pneumonia is the most commonly described infection.
  • Other bacterial infections associated with Sweet syndrome also include those due to Salmonella or Staphylococcus species, Yersinia enterocolitica, Entamoeba coli, Helicobacter pylori, Borrelia burgdorferi, nontuberculous organisms, (atypical), and Tuberculous mycobacteria.
  • Sweet syndrome may be a presenting feature of coccidiomycosis (Dicaudo, 2005). Viral agents such as HIV, cytomegalovirus (CMV), hepatitis A, and hepatitis B have also been implicated.
  • Yersinia-associated Sweet syndrome has been noted to improve with antibiotics.
• Multiple drugs have been reported to cause Sweet syndrome.
• • Some of these reactions have been noted in patients with underlying malignancy; therefore, the validity of these possible associations is unclear.
  • Because the dominant cell in the dermal infiltrate is a neutrophil, drug-induced Sweet syndrome is not considered to be a drug hypersensitivity.
  • G-CSF is a well-established factor.
  • Established factors include trimethoprim-sulfamethoxazole (Bactrim), all-trans retinoic acid, and minocycline, which have all appeared in more than 1 case report.
  • Anecdotal or limited reports of drug or device associations include lithium, furosemide, hydralazine, carbamazepine, oral contraceptives, the Mirena intrauterine device, COX-2 inhibitors, doxycycline, diazepam, diclofenac, nitrofurantoin, propylthiouracil, lenalidomide, bortezomib, abacavir, imitinib and vaccinations (eg, for bacille Calmette-Guérin, smallpox, pneumococcal organisms).
• Systemic disorders
• • Associated inflammatory disease can be identified in about 15% of patients with Sweet syndrome.
  • The most common associated diseases are Crohn disease and ulcerative colitis, which some authors consider part of a continuum of neutrophilic dermatosis.
  • Sjögren syndrome, Behçet disease, lupus erythematosus, rheumatoid arthritis, and undifferentiated connective tissue disease have been reported in association with acute neutrophilic dermatitis.
• Miscellaneous
• • Rare cases of acute neutrophilic dermatitis have occurred with spinal surgery, sarcoidosis, erythema nodosum, relapsing polychondritis, or thyroiditis.
  • A few cases have been observed during pregnancy.
  • Several cases of Sweet syndrome occurred with polycythemia vera.
  • One patient had a mutation in the prothrombin gene (G20210A), but no conclusive association can be made at this time.
• Diagnostic criteria
• • The presence of 2 major and 2 minor clinical findings have been proposed as criteria for diagnosis, as suggested by Su and Liu and revised by von den Driesch.
  • Major criteria
    • Abrupt onset of tender or painful erythematous plaques or nodules, occasionally with vesicles, pustules, or bullae
    • Predominantly neutrophilic infiltration in the dermis without leukocytoclastic vasculitis
  • Minor criteria
    • Preceding nonspecific respiratory or gastrointestinal tract infection or vaccination or associated with inflammatory disease, hemoproliferative disorders, solid malignant tumors, or pregnancy
    • Periods of general malaise and fever (body temperature >38°C)
    • Laboratory values during onset showing a erythrocyte sedimentation rate >20 mm, positive C-reactive protein (CRP) result, segmented nuclear neutrophils, bands >70% in peripheral blood smears, and leukocytosis (count >8000/µL) (meeting 3 of 4 of these values is necessary)
    • Excellent response to treatment with systemic corticosteroids or potassium iodide

DIFFERENTIALS

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Other Problems to be Considered

Bowel-associated dermatitis-arthritis syndrome
Neutrophilic rheumatoid dermatitis
Leukocytoclastic vasculitis
Acral erythema
Leukemia cutis
Acute hemorrhagic edema of childhood

WORKUP

Section 5 of 11  

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Lab Studies

• The diagnosis is usually based on histopathologic examination by a qualified dermatopathologist, but the laboratory findings are nonspecific. Clinicopathologic correlation is important because the bowel bypass syndrome may present with skin lesions with an identical histologic picture.
• Several tests are helpful.
• • A CBC count with differential must be ordered to screen for underlying hematologic disorders.
  • Neutrophilia is typically present, but the absence of neutrophilia in a patient who is neutropenic does not rule out Sweet syndrome.
  • Anemia and thrombocytopenia are common in patients with underlying malignancy.
  • Abnormalities in the CBC count should prompt consideration of bone marrow biopsy.
• The erythrocyte sedimentation rate (ESR) and CRP level should be determined. The ESR is elevated in more than 90% of cases, and the C-reactive protein value also may be elevated. However, both of these findings are nonspecific manifestations of inflammation.
• • Urinalysis may show proteinuria and/or hematuria.
  • On a hepatic panel, concentrations of hepatic enzymes may be elevated nonspecifically.
  • Antineutrophilic cytoplasmic antibodies (ANCAs) have been described but not consistently found in all patients with Sweet syndrome.
  • Lesions should be cultured for bacteria, fungi, and mycobacteria to rule out infection.

Imaging Studies

• A chest radiograph should be obtained if pulmonary symptoms are present because lung involvement may occur and is responsive to systemic corticosteroids.
• Sweet syndrome is the presenting sign of malignancy in approximately two thirds of the cases of malignancy-associated Sweet syndrome.
• The presence of ulcerative lesions, oral lesions, abnormal platelet counts, or anemia should prompt investigation for an underlying malignancy.
• Some authors recommend a directed systemic evaluation in all patients with Sweet syndrome.
• If an underlying malignancy is suspected, the appropriate imaging modality should be used for early detection and treatment.
• 2-[fluorine 18]-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) is especially useful in evaluating myeloproliferative disorders, but they can also be helpful in assessing some solid tumors and has successfully depicted early malignancies.

Procedures

• Skin biopsy should be performed to confirm the diagnosis.
• Bone marrow aspiration is indicated if CBC count is abnormal, and it should be considered in all cases of atypical bullous or ulcerative Sweet syndrome.
• Age-appropriate cancer screening and evaluation for inflammatory bowel disease are indicated if no other underlying cause is found, especially in patients with bullous or ulcerative lesions.

Histologic Findings

The classic histopathologic pattern consists of a dense, diffuse neutrophilic infiltrate in the reticular dermis. Leukocytoclastic nuclear debris is typically present interstitially, and massive papillary dermal edema is common. True vasculitic changes (expansion of postcapillary venule wall with fibrin deposition) are typically absent, though subtle vasculitic changes may occur. Eosinophils and lymphocytes are present in some instances, but neutrophils usually predominate.

The epidermis usually is spared, though nonspecific findings such as spongiosis and subcorneal pustule formation can be seen.

Results of direct immunofluorescence testing are noncontributory. In rare cases, the inflammation extends to involve the subcutis. In essentially all instances, cases with subcutaneous involvement also show extensive involvement of the reticular dermis.

Vasculitis is seen in cases of neutrophilic dermatoses of the dorsal hand (NDDH). As mentioned earlier, this is a controversial diagnosis, and whether this entity should be included as an anatomically limited version of Sweet syndrome or whether it should be categorized as a primary vasculitis is unclear.

TREATMENT

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Medical Care

• In most cases, prednisone is extremely and rapidly effective, in doses of 40-80 mg/d.
• • Pulmonary infiltrates also tend to respond promptly to prednisone.
  • However, despite the initial excellent response, recurrences of neutrophilic dermatitis are common and generally develop as steroid use is being tapered. If the underlying disease flares, it may take longer to effectively taper therapy.
  • High-potency topical steroids (eg, clobetasol propionate 0.05%) or intralesional glucocorticoids (eg, triamcinolone acetonide 3.0-10 mg/mL) may also be useful in localized lesions.
• For long-term management, numerous drugs may be helpful. Many of the medications work by inhibiting neutrophil chemotaxis, but none have been shown to be better than corticosteroids.
• • Indomethacin, colchicine, and potassium iodide were helpful in small series of patients.
  • Dapsone, cyclosporine, etretinate, pentoxifylline, and clofazimine also have been used, with anecdotal success.
  • Doxycycline, metronidazole, isotretinoin, methotrexate, cyclophosphamide, chlorambucil, pulse doses of methylprednisolone, and interferon alpha are also reportedly successful.
• If an underlying cause can be identified, it should be treated, eg, by means of resection of solid tumors, treatment of infections, and discontinuation of causative medication. Successful therapy of the underlying disorder may promote resolution of Sweet syndrome and prevent recurrences.
• Etanercept has been reported to control the skin manifestations of Sweet syndrome in a small case series of rheumatoid arthritis patients (Yamaguchi, 2006).
• In the pediatric population, long-term use of corticosteroids can cause problems with linear growth, blood pressure, and blood glucose levels. Children may also have social sequelae associated with their use. Therefore, attempts are usually made to treat children with steroid-sparing drugs.

Consultations

• Consultation with a dermatologist is indicated for the diagnosis and evaluation of underlying causes.
• An internal medicine specialist may be consulted to evaluate any underlying or triggering diseases.

MEDICATION

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The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Drug Category: Corticosteroids

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Drug Category: Anti-inflammatories

These agents modulate events leading to inflammatory reactions.

FOLLOW-UP

Section 8 of 11  

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Further Outpatient Care

• Outpatient care of the skin lesions is usually coordinated by a dermatologist in conjunction with other physicians who may be involved if other underlying diseases are present.

Deterrence/Prevention

• Deterrence is based on avoiding known triggers, such as medications, and treatment of underlying conditions that may be factors.

Complications

• Some lesions resolve without scarring, though pigmentary changes may take months to fade.

Prognosis

• The outcome depends on the underlying condition, but recurrence may occur in up to 50% of patients and is most likely in cases associated with hematologic malignancy or drug reactions.

Patient Education

• Patient education should include information about the variable course of this condition, as well as advice on self-monitoring for signs and symptoms of other diseases.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Failure to evaluate for associated underlying conditions is the most likely reason for medical/legal problems.

Special Concerns

• Underlying disease
• • It is important to recognize and treat any associated or underlying systemic diseases or malignancies.
  • Sweet syndrome may be a clue to the diagnosis of a systemic disorder or malignancy, and important clues should not be ignored.
  • Imaging such as ultrasonography, CT, PET, or MRI may be helpful in identifying underlying malignancies.
• Pregnancy
• • Acute neutrophilic dermatosis can occur during pregnancy and may recur in subsequent pregnancies.
  • Presently, it is believed that the disease is not associated with fetal harm.

MULTIMEDIA

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Media file 1:  Red nodules and plaques on the lateral aspect of the hand.
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Media type:  Photo

Media file 2:  Multiple lesions on the scalp.
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Media file 3:  Close-up of a scalp lesion.
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REFERENCES

Section 11 of 11

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• Saliba WR, Goldstein LH, Habib GS, Elias MS. Sweet's syndrome affecting the external auditory canal and tympanic membrane. J Laryngol Otol. Jan 2004;118(1):48-9. [Medline].
• Smith KJ, Skelton H. Acute onset of neutrophilic dermatosis in patients after therapy with a COX-2-specific inhibitor. Int J Dermatol. May 2003;42(5):389-93. [Medline].
• Su WP, Liu HN. Diagnostic criteria for Sweet''s syndrome. Cutis. Mar 1986;37(3):167-74. [Medline].
• Tursen U, Bocekli E, Kaya TI, et al. Sweet''s syndrome in a woman with a prothrombin gene (G20210A) mutation. Int J Dermatol. Sep 2002;41(9):596-7. [Medline].
• Vignon-Pennamen MD, Aractingi S. Sweet''s syndrome and leukemia cutis: a common skin homing mechanism?. Dermatology. 2003;206(2):81-4. [Medline].
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Article Last Updated: Mar 7, 2007