eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Acute Febrile Neutrophilic Dermatosis

Author: Yoon-Soo Bae-Harboe, MD, General Surgery Intern, New York-Presbyterian Hospital of Weill Cornell Medical College
Coauthor(s): Sharon A Salter, MD, Staff Physician, Department of Psychiatry, Tufts-New England Medical Center; Alexa F Boer Kimball, MD, MPH, Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital
Contributor Information and Disclosures

Updated: Jul 31, 2009

Introduction

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.1

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.1,2 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.1,3,4,5

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.1,6,7,8

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. However, recent reports suggest that vasculitis should not exclude the diagnosis since it has been shown to occur in many patients with Sweet’s syndrome, which may represent an epiphenomenon instead of a primary immune-mediated process.9

Pathophysiology

Acute febrile neutrophilic dermatosis (Sweet syndrome) is a reactive process (a hypersensitivity reaction) that occurs in response to systemic factors, such as hematologic disease, infection, inflammation, vaccination, or drug exposure.10 The condition is neutrophil mediated, as evidenced by its histopathologic appearance, associated neutrophilia, and response to medications that affect neutrophil activity.1

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. The functional properties of neutrophils, rather than the absolute number, is thought to be significant because patients with Sweet syndrome due to G-CSF develop lesions as the neutrophil count rapidly increases, despite the absolute neutrophil count being low.7,11,12  

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.8

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.13

Frequency

International

Acute febrile neutrophilic dermatosis (Sweet syndrome) is uncommon but not rare. Several hundred cases have been reported in the literature. In several series, 10 -20% of cases have occurred in a setting of malignancy, though most are idiopathic or associated with benign conditions.5,6

Mortality/Morbidity

Most cases of acute febrile neutrophilic dermatosis (Sweet syndrome) 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.4

Race

Acute febrile neutrophilic dermatosis (Sweet syndrome) has no known racial predilection.1

Sex

The common reactive variant of acute febrile neutrophilic dermatosis (Sweet syndrome) does have a female predominance, with a female-to-male ratio of 15:1.14 However, this predilection was not noted in series of cases associated with malignancy.15 No sex predisposition has been observed in children.16

Age

Typically, women with acute febrile neutrophilic dermatosis (Sweet syndrome) are aged 30-50 years.1 However, cases in neonates as young as 5 days have been described.17 In children, Sweet syndrome is extremely rare and generally associated with infection.16

Clinical

History

  • Fever typically precedes the appearance of each crop of lesions in acute febrile neutrophilic dermatosis (Sweet syndrome). The fever can precede the skin disease by several days to weeks; however, it may also occur simultaneously.1
  • The crop of plaques or nodules in the classic form often appears abruptly and may persist for weeks or several months (days to weeks) if untreated.1,3
  • 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.3,10
  • Headache, malaise, and arthralgias are common.1,18

Physical

  • Skin manifestations of acute febrile neutrophilic dermatosis (Sweet syndrome)
    • 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.1,3,6,18
    • Plaques can cause pain and burning, but they are not pruritic. Lesions spontaneously resolve without scarring, or they resolve after treatment.1
    • The face, neck, and extremities primarily are affected, characteristically in an asymmetric distribution.1
    • 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.19,20
    • 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.3
    • Lesions on the dorsum of the hand are not uncommon. They sometimes appear vasculitic, which is not typically seen in Sweet syndrome.18
      • 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.18
      • Some believe that this is an anatomically limited form of Sweet syndrome, whereas others categorize this as a primary vasculitis.9
      • 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.21
      • The vasculitis does not appear to be a primary immune-mediated process, as is seen in the primary leukocytoclastic vasculitides; 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.9


Red nodules and plaques on the lateral aspect of ...

Red nodules and plaques on the lateral aspect of the hand.

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Red nodules and plaques on the lateral aspect of ...

Red nodules and plaques on the lateral aspect of the hand.


Multiple lesions on the scalp.

Multiple lesions on the scalp.

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Multiple lesions on the scalp.

Multiple lesions on the scalp.


Close-up of a scalp lesion.

Close-up of a scalp lesion.

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Close-up of a scalp lesion.

Close-up of a scalp lesion.

 

  • Mucosal lesions of acute febrile neutrophilic dermatosis (Sweet syndrome)
    • 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.7
    • 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, iritis, and sudden visual loss.22,23,24
  • Extracutaneous manifestations of acute febrile neutrophilic dermatosis (Sweet syndrome)
    • Sweet syndrome can involve several organ systems.1
    • 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.25,26,27
    • Other extracutaneous sites that have been reported include the bones, intestines, joints, bone marrow, liver, heart, muscles, spleen, and kidneys.1,28,29,30,31,32,33,34,35,36
    • 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.37
      • HLA types B54 and CW1 are associated with Sweet syndrome with CNS involvement in Japanese patients.37
      • Unlike Behçet syndrome, in which CNS involvement is progressive and severe, Sweet syndrome usually causes transient CNS involvement, but recurrences may occur.37
    • Proteinuria, hematuria, and decreased creatinine clearance have been reported.15
    • Cerebrospinal fluid pleocytosis also has been described, as has a sterile chronic recurrent multifocal osteomyelitis in children.38
  • 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.39
    • The lesions may also be photodistributed or localized to the site of a previous phototoxic reaction (eg, sunburn).1,39

Causes

Potential causes of acute febrile neutrophilic dermatosis (Sweet syndrome) are numerous, but some associations are well documented.4 Classic or idiopathic Sweet syndrome is the most common presentation and accounts for more than 50% of cases.40 Sweet syndrome associated with a (malignant) neoplasm accounts for approximately 11-54% of the cases. Most of these are hematopoietic malignancies (most commonly acute myeloid leukemia), but some 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.4,40

  • Hematologic malignancy
    • Myelodysplasia and chronic myelogenous leukemia may be associated with Sweet syndrome.15,41 Sweet syndrome can also be seen in association with acute myeloid leukemia (AML), including the promyelocytic (M3) variant of AML.15,42
    • 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).31,43
    • Nonhematologic malignancy has been associated with Sweet syndrome; rates of genitourinary, breast, and gastrointestinal cancers appear to be slightly increased in this group.15
  • Nonhematologic malignancy: Other rarely reported associations include osteosarcoma, oral cancer/tonsil cancer, ovarian cancer, thyroid cancer, lung cancer, pheochromocytoma, and rectal carcinoma.8
  • Infection
    • Multiple infections are described in association with Sweet syndrome, often involving the upper respiratory tract. Streptococcal pneumonia is the most commonly described infection.39
    • 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), Tuberculous mycobacteria, Mycobacterium chelonae, and Penicillium species.39,44
    • Sweet syndrome may be a presenting feature of coccidiomycosis.26 Viral agents such as HIV, cytomegalovirus (CMV), hepatitis A, and hepatitis B has also been implicated.45
    • Yersinia -associated Sweet syndrome has been noted to improve with antibiotics.46
  • Drugs
    • Multiple drugs have been reported to cause Sweet syndrome, with some of these reactions being noted in patients with underlying malignancy; therefore, the validity of these possible associations is unclear.1
    • 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.1,47
    • Established factors include trimethoprim-sulfamethoxazole (Bactrim), all-trans retinoic acid, and minocycline, which have all appeared in more than 1 case report.5
    • Anecdotal or limited reports of drug or device associations include lithium, furosemide, hydralazine, carbamazepine, oral contraceptives, the Mirena intrauterine device, COX-2 inhibitors, azathioprine, doxycycline, diazepam, diclofenac, nitrofurantoin, propylthiouracil, lenalidomide, bortezomib, abacavir, imitinib and vaccinations (eg, for bacille Calmette-Guérin, smallpox, pneumococcal organisms, influenza).10,34,48,49,50,51,52,53
  • Systemic disorders
  • Miscellaneous
  • Diagnostic criteria (proposed by Su and Liu and revised by von den Driesch): The presence of 2 major and 2 minor clinical findings have been proposed as criteria for diagnosis.
    • 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
  • Diagnostic criteria for drug-induced Sweet syndrome (proposed by Walker et al): All 5 criteria below are required for the diagnosis of drug-induced Sweet syndrome.
    • Drug eruption
      • Abrupt onset of painful erythematous plaques or nodules
      • Histopathologic evidence of a dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis
      • Pyrexia (38°C)
      • Temporal relationship between drug ingestion and clinical presentation, or temporally related recurrence after oral challenge
      • Temporally related resolution of lesions after drug withdrawal or treatment with systemic corticosteroids5

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References
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Further Reading

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Keywords

acute febrile neutrophilic dermatosis, Sweet syndrome, Sweet disease, Sweet's syndrome, Sweet's disease, neutrophilic dermatitis, Gomm-Button disease, granulocyte colony-stimulating factor, G-CSF

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Contributor Information and Disclosures

Author

Yoon-Soo Bae-Harboe, MD, General Surgery Intern, New York-Presbyterian Hospital of Weill Cornell Medical College
Yoon-Soo Bae-Harboe, MD is a member of the following medical societies: American Medical Student Association/Foundation
Disclosure: Nothing to disclose.

Coauthor(s)

Sharon A Salter, MD, Staff Physician, Department of Psychiatry, Tufts-New England Medical Center
Sharon A Salter, MD is a member of the following medical societies: Alpha Omega Alpha and American College of Obstetricians and Gynecologists
Disclosure: Nothing to disclose.

Alexa F Boer Kimball, MD, MPH, Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital
Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco
Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Apsara Consulting fee Independent contractor

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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