Acute Febrile Neutrophilic Dermatosis Workup

  • Author: Yoon-Soo Cindy Bae-Harboe, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 10, 2012
 

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, as follows:

  • 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]

Other findings may be as follows:

  • Urinalysis may show proteinuria and/or hematuria.[15]
  • On a hepatic panel, concentrations of hepatic enzymes may be elevated nonspecifically.[45]
  • Antineutrophilic cytoplasmic antibodies (ANCAs) have been described but not consistently found in all patients with Sweet syndrome.[79]
  • Lesions should be cultured for bacteria, fungi, and mycobacteria to rule out infection.[1]
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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.[46] 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.[80]

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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]

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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.

Histiocytoid Sweet syndrome is a variant that has been described and includes histiocytoid cells, which are immature myeloid cells commonly mistaken as histiocytes. The most important differential diagnosis for this variant is leukemia cutis.[81]

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Contributor Information and Disclosures
Author

Yoon-Soo Cindy Bae-Harboe, MD  Resident Physician, Department of Dermatology, Boston University School of Medicine, Boston Medical Center

Yoon-Soo Cindy 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.

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

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

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

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: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Christina N Alavian, MD, Resident Physician, Division of Dermatology, University of Massachusetts Medical School

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.

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Red nodules and plaques on the lateral aspect of the hand.
Multiple lesions on the scalp.
Close-up of a scalp lesion.
 
 
 
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