Original article
Sweet syndrome in patients with and without malignancy: A retrospective analysis of 83 patients from a tertiary academic referral center

Portions of this work were presented at the Annual Meeting of the Medical Dermatology Society in Orlando, FL, on March 2, 2017, and the 2017 Annual Meeting of the Society of Investigative Dermatology in Portland, OR on April 26-29, 2017. This work has not been previously published.
https://doi.org/10.1016/j.jaad.2017.09.013Get rights and content

Background

Sweet syndrome is a neutrophilic dermatosis that may be categorized into classic, malignancy-associated, and drug-induced subtypes. Few studies have systematically analyzed this rare disorder.

Objective

To describe the clinicopathologic characteristics and treatment of Sweet syndrome and identify characteristics associated with concurrent malignancy.

Methods

We retrospectively reviewed patients with Sweet syndrome at the University of Pennsylvania from 2005 to 2015.

Results

We identified 83 patients (mean age, 57 years; 51% male) with Sweet syndrome: 30% with the classic form, 44% with the malignancy-associated form, 24% with the drug-induced form in the setting of malignancy, and 2% with the drug-induced form. Acute myeloid leukemia was the most common malignancy (in 24 of 83 patients [29%]). Filgrastim was the most common medication (used in 8 of 83 patients [10%]). Leukopenia (P < .001), anemia (P = .002), thrombocytopenia (P < .001), absence of arthralgia (P < .001), and histiocytoid or subcutaneous histopathology (P = .024) were associated with malignancy (χ2 test).

Limitations

This was a retrospective study that represents patients from a single tertiary academic referral center, which may limit its generalizability to other settings.

Conclusion

When caring for patients with Sweet syndrome, dermatologists should be aware of the potential association of leukopenia, anemia, thrombocytopenia, absence of arthralgia, and histiocytoid or subcutaneous histopathology with malignancy.

Section snippets

Study design, setting, and participants

We performed a retrospective study of adults with Sweet syndrome seen at the Hospital of the University of Pennsylvania between 2005 and 2015. After approval by the institutional review board, we searched the medical records for patients with a possible diagnosis of Sweet syndrome. Of 524 patients, 441 did not meet inclusion criteria (Fig 2). The medical records of the remaining 83 patients were reviewed and confirmed to satisfy modified Su and Liu diagnostic criteria2, 5 for Sweet syndrome.

Data collection

Patient characteristics

The mean age of presentation of Sweet syndrome in our cohort was 57 years; 51% of patients were male. Of the 83 patients, 69 (83%) were white, 8 (10%) were black or African American, and 2 (2%) were Hispanic or Latino; 58 (70%) presented in the inpatient setting.

Clinicopathologic features in patients with and without concurrent malignancy

Table II compares Sweet syndrome in patients with and without concurrent malignancy. Leukopenia (P < .001), anemia (P = .002), thrombocytopenia (P < .001), and absence of arthralgia (P < .001) were clinical features that were more

Discussion

Our results corroborate a handful of analyses identifying anemia,8, 9, 10, 11 thrombocytopenia,8, 11 and absence of arthralgia11 as clinical features occurring more often in patients with Sweet syndrome with concurrent malignancy. We also found an increased frequency of leukopenia, which was notably more common in our cohort than leukocytosis was. Anemia was present in a majority of patients both with and without malignancy, whereas leukopenia and arthralgia were present in a minority of

Conclusion

Our retrospective analysis, which is one of the largest published to date, underscores the importance of malignancy evaluation in Sweet syndrome. Leukopenia, anemia, thrombocytopenia, absence of arthralgia, and histiocytoid or subcutaneous histopathology were associated with malignancy in our cohort. Dermatologists should be aware of these potential associations with malignancy when caring for patients with Sweet syndrome.

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    Supported by a private donation from Mr Jerry and Mrs Joan Berstein. The sponsors had no role in the design and conduct of the study; in the collection, analysis, and interpretation of data; or in the preparation, review, and approval of the manuscript.

    Conflicts of interest: None declared.

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    © 2017 by the American Academy of Dermatology, Inc.