Sweet syndrome associated with malignancies: A retrospective analysis of 25 patients from West China hospital

Abstract

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is a rare inflammatory disorder characterized by sudden onset of variably tender brightly erythematous‐to‐violaceous papules, plaques, or nodules. To clarify the frequency, clinical course, therapeutic response, and outcome of Sweet syndrome with malignancy. We conducted a retrospective review of patients diagnosed with SS between 2009 and 2019 with a diagnosis of Sweet syndrome in West China hospital. Records were analyzed for variables, clinical features, etiological factors, laboratory results, and treatment outcomes. The 25 cases under study consisted of 14 (56%) females and 11 (44%) males. Mean age was 52.2 years (range: 4‐76 years). Nodules were the commonest lesion presentation (48%), involving mostly the lower limbs (52%). Pain of lesion was identified as the most common symptom (88%), followed by fever (76%) and cough (44%). Among the patients, 52% exhibited the classic form, 48% showed the malignancy‐associated form, and 4% exhibited the drug‐induced form in the setting of malignancy. Acute myeloid leukemia was the most common malignancy, observed in 5 of 12 patients (41.6%). Anemia (P = .002) was associated with malignancy (Chi‐square test).

New Practical Aspects of Sweet Syndrome

“…non-malignancy-associated SS as well as malignancy associated SS [6][7][8]. SS has also been reported to have no racial predilection, although neuro-Sweet disease, a variant of SS presenting with neurological symptoms, has been most commonly noted in Japanese patients [9].…”

“…SS has also been recently associated with a number of solid organ malignancies including cervical cancer [118], cholangiocarcinoma [119], colorectal cancer [111], gastric adenocarcinoma [8], and lung cancer [8]. Thus, SS may be considered a rare cutaneous paraneoplastic syndrome associated with a host of malignancies.…”

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) in Acute Myeloid Leukemia Patients: A 28-Year Institutional Experience

“…Sweet syndrome (SS) is a neutrophilic dermatosis characterized by the abrupt eruption of painful edematous and erythematous papules. While it can be idiopathic or associated with autoimmune or inflammatory conditions and medication exposure, between 10% and 20% of all SS cases are associated with underlying malignancy [1][2][3][4][5][6][7]; it is particularly well known to be associated with hematologic malignancies, most commonly leukemias and myelodysplastic syndromes/myeloproliferative neoplasms (MDSs/ MPNs) (MDS+/−MPN). While cases among patients with MDS and hairy cell leukemia have been described, the association between SS and acute myeloid leukemia (AML) has been most extensively studied [6].…”

Transformation of a myelodysplastic syndrome to acute myeloid leukemia and concurrent necrotizing sweet syndrome

“…2 Some of the clinical conditions related to this syndrome include infections, autoimmune diseases, inflammatory bowel diseases, vaccines, pregnancy, certain medications, neoplasms and idiopathic. 3 Paraneoplastic syndromes are clinical clues that neoplasms cause in places outside their primary location and that are directly associated with them or with their metastases. They may occur accompanying an established cancer or as the first sign of malignancy or its recurrence.…”

“… 2 Some of the clinical conditions related to this syndrome include infections, autoimmune diseases, inflammatory bowel diseases, vaccines, pregnancy, certain medications, neoplasms and idiopathic. 3 …”