Sweet’s Syndrome Successfully Treated with Granulocyte and Monocyte Adsorption Apheresis

Abstract

Sweet’s syndrome is a neutrophilic dermatosis characterized by an abrupt onset of painful erythematous lesions showing neutrophilic infiltrates in the dermis. Fever and an elevated neutrophil level are generally observed. Sweet’s syndrome may be idiopathic, malignancy-associated, or drug-induced (mainly involving granulocyte colony-stimulating factor (G-CSF) administration). Although systemic corticosteroids are usually effective, the symptoms of Sweet’s syndrome recur in some refractory cases. Herein, we report a case of a 55-year-old Japanese woman with recurrent symptoms of fever (>39°C) and painful erythematous lesions on her four extremities, trunk, and neck. Laboratory findings revealed leukocytosis and high levels of C-reactive protein (CRP) and G-CSF. She was diagnosed with a recurrence of Sweet’s syndrome, and was exclusively treated with granulocyte and monocyte adsorption apheresis (GMA) therapy once a week for 3 consecutive weeks. After the first session of GMA therapy, all symptoms including the erythematous lesions and fever were completely resolved, and serum G-CSF level was reduced. Leukocyte count, neutrophil count, serum amyloid A protein, and CRP levels were restored within normal ranges by 2 weeks. Thus, GMA therapy can successfully treat a patient with recurrent Sweet’s syndrome, potentially related to the restoration of elevated serum G-CSF levels.

Insights Into the Pathogenesis of Sweet's Syndrome

“…With advances in our understanding of the pathophysiology of neutrophilic dermatoses, especially the role of TNF-α and IL-1β, the use of targeted therapy with IL-1 and TNF-α inhibitors has been effective ( 323 , 593 , 597 – 603 ). There have been reports of several novel treatments for SS, including granulocyte and monocyte adsorption apheresis, but due to the rarity of SS and the effectiveness of established treatments there have been limited investigations into these alternative treatments ( 604 ).…”

Acute febrile neutrophilic dermatosis in a patient with Crohn’s disease: case report and review of the literature

“…According to the literature, in patients with a refractory form of the syndrome, treatment with potassium iodide, colchicine, indomethacin, clofazimine, cyclosporine, and dapsone has also been used successfully (10). Recently, a patient with this syndrome was successfully treated with granulocyte and monocyte adsorption apheresis (14). After treatment or spontaneous remission, depending on the underlying disease, the syndrome usually has a good prognosis.…”

Use of granulocyte and monocyte adsorption apheresis in dermatology (Review)

“…GMA may be a useful non-pharmacologic tool for SS, with no safety concerns. Fujii et al (75) reported a case of a 55-year-old woman affected by SS, previously treated with PSL, nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine, who underwent GMA for three times, once a (76). At present, AOSD therapeutic strategy aims to prevent organ damage and life-threatening complications and minimize adverse effects of treatment.…”