A Case of Malignancy-associated Histiocytoid Sweet Syndrome in a Patient with AML / 대한피부과학회지

Sweet syndrome is a disorder characterized by painful, erythematous, cutaneous plaques and nodules of rapid onset accompanied by fever, leukocytosis, and neutrophilia. We encountered a case of a recurrent histiocytoid Sweet syndrome in a patient with acute myeloid leukemia (AML). A 64-year-old Korean man was diagnosed with complete remission of AML and had symptomatic nodules and plaques on the dorsal sides of both hands. Approximately 3 years prior, he also had symptomatic plaques at the same site and had been diagnosed with MDS (Myelodysplastic syndrome). We performed a biopsy and diagnosed this case as a malignancy-associated histiocytoid Sweet syndrome. Most Sweet syndrome cases are acute; in contrast, this case was chronic with a relapse. In addition, histopathologic examination showed a dense histiocytic infiltration. These histiocytoid cells are usually misinterpreted as histiocytes; however, they are actually immature myeloid cells. Herein, we report a case of a recurrent malignancy-associated histiocytoid Sweet syndrome in a patient with a hematologic disorder.

Clinical Study of 24 Cases with Sweet's Syndrome / 대한피부과학회지

BACKGROUND: Sweet's syndrome is a neutrophilic dermatosis that has various clinical characteristics and is often associated with inflammatory, malignant or autoimmune diseases, so the viral or bacterial antigens and tumor antigens are suspected to play a role in the pathogenesis of Sweet's syndrome. OBJECTIVE: This study was conducted to obtain a better understanding of the clinical features of Sweet's syndrome and estimate the prevalence of malignant associated Sweet's syndrome in Korea. METHODS: Clinical manifestations and histopathologic features of 24 Sweet's syndrome patients were evaluated. RESULTS: The age was varied from 24 to 85 years, averaging 49.3 years. The male to female ratio was 1:2.4 . The site of predilection was the extremities, and the most common involved site was the upper extremities. The associated clinical symptoms were fever, arthralgia, myalgia, and oral ulcer in varying frequencies. In laboratory examinations, there were increased ESR, leukocytosis, and neutrophilia in part of the patients. The associated systemic diseases were found in 58.3% of the patients and hematologic malignancies were the most common with prominent AML-association. The majority of the patients were treated with oral or topical steroids and the recurrences were reported in 50%. CONCLUSION: The associated systemic diseases were presented in 58.3% of 24 Sweet's syndrome patients. And there was high association of Sweet's syndrome and hematologic malignancies, especially AML, as previously reported, which implies the importance of thorough investigation for underlying diseases in the Sweet's syndrome patients.