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Acute Febrile Neutrophilic Dermatosis: Differential Diagnoses & Workup

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

Differential Diagnoses

Behcet Disease
Drug Eruptions
Erythema Multiforme
Erythema Nodosum
Herpes Simplex
Pyoderma Gangrenosum

Other Problems to Be Considered

Bowel-associated dermatitis-arthritis syndrome
Neutrophilic rheumatoid dermatitis
Leukocytoclastic vasculitis
Acral erythema
Leukemia cutis
Acute hemorrhagic edema of childhood
Halogenoderma
Rosacea fulminans4

Workup

Laboratory Studies

  • The diagnosis of acute febrile neutrophilic dermatosis (Sweet syndrome) 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.1,3,4
  • 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.1,3,18
  • 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.1,3
    • Urinalysis may show proteinuria and/or hematuria.15
    • On a hepatic panel, concentrations of hepatic enzymes may be elevated nonspecifically.39
    • Antineutrophilic cytoplasmic antibodies (ANCAs) have been described but not consistently found in all patients with Sweet syndrome.60
    • Lesions should be cultured for bacteria, fungi, and mycobacteria to rule out infection.1

Imaging Studies

  • A chest radiograph should be obtained if pulmonary symptoms of acute febrile neutrophilic dermatosis (Sweet syndrome) are present because lung involvement may occur and is responsive to systemic corticosteroids.4
  • Sweet syndrome is the presenting sign of malignancy in approximately two thirds of the cases of malignancy-associated Sweet syndrome.4
  • The presence of ulcerative lesions, oral lesions, abnormal platelet counts, or anemia should prompt investigation for an underlying malignancy.15
  • Some authors recommend a directed systemic evaluation in all patients with Sweet syndrome.40
  • If an underlying malignancy is suspected, the appropriate imaging modality should be used for early detection and treatment.1
  • 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.61

Procedures

  • Skin biopsy should be performed to confirm the diagnosis of acute febrile neutrophilic dermatosis (Sweet syndrome).1
  • 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.4
  • 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.1

Histologic Findings

The classic histopathologic pattern of acute febrile neutrophilic dermatosis (Sweet syndrome) 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.1

The epidermis usually is spared, though nonspecific findings such as spongiosis and subcorneal pustule formation can be seen.3

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.3

Vasculitis is seen in cases of neutrophilic dermatoses of the dorsal hand.18 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.

More on Acute Febrile Neutrophilic Dermatosis

Overview: Acute Febrile Neutrophilic Dermatosis
Differential Diagnoses & Workup: Acute Febrile Neutrophilic Dermatosis
Treatment & Medication: Acute Febrile Neutrophilic Dermatosis
Follow-up: Acute Febrile Neutrophilic Dermatosis
Multimedia: Acute Febrile Neutrophilic Dermatosis
References
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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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