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

2017 ◽  
Vol 9 (2) ◽  
pp. 13-18 ◽  
Author(s):  
Asami Fujii ◽  
Yoko Mizutani ◽  
Yuki Hattori ◽  
Tomoko Takahashi ◽  
Hidenori Ohnishi ◽  
...  

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.

2008 ◽  
Vol 22 (3) ◽  
pp. 296-298 ◽  
Author(s):  
Massud Ali ◽  
Donald R Duerksen

A 47-year-old man with a history of ulcerative colitis on prednisone and azathioprine was admitted to the hospital with a four-day history of fever, skin rash, arthralgias and leukocytosis. A skin biopsy demonstrated neutrophilic infiltration of the dermis that was consistent with Sweet’s syndrome. He improved after several days with an increase in his prednisone and azathioprine. Sweet’s syndrome is a rare cutaneous manifestation of inflammatory bowel disease, with approximately 40 cases reported in the literature. In a previously reported case of a patient with ulcerative colitis-associated Sweet’s syndrome who was on azathioprine at the time of the skin eruption, the azathioprine was stopped, raising the possibility of drug-induced Sweet’s syndrome. In the present case, the azathioprine was actually increased with complete resolution of the skin manifestations. This would support the theory that immunosuppressive therapy is the mainstay of therapy for this condition. In conclusion, Sweet’s syndrome is a neutrophilic dermatosis that is rarely associated with ulcerative colitis. It may occur while on immunosuppressive therapy and responds to an intensification of immunosuppression.


2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
J. L. Barton ◽  
L. Pincus ◽  
J. Yazdany ◽  
N. Richman ◽  
T. H. McCalmont ◽  
...  

Sweet's syndrome is an acute febrile neutrophilic dermatosis which usually presents as an idiopathic disorder but can also be drug induced, associated with hematopoetic malignancies and myelodysplastic disorders, and more, infrequently, observed in autoimmune disorders. Sweet's syndrome has been reported in three cases of neonatal lupus, three cases of hydralazine-induced lupus in adults, and in nine pediatric and adult systemic lupus erythematosus (SLE) patients. We describe three additional adult cases of Sweet's associated with SLE and provide a focused review on nondrug-induced, nonneonatal SLE and Sweet's. In two of three new cases, as in the majority of prior cases, the skin rash of Sweet's paralleled underlying SLE disease activity. The pathogenesis of Sweet's remains elusive, but evidence suggests that cytokine dysregulation may be central to the clinical and pathological changes in this condition, as well as in SLE. Further research is needed to define the exact relationship between the two conditions.


2019 ◽  
Vol 7 ◽  
pp. 232470961989516
Author(s):  
Arash Mollaeian ◽  
Hadi Roudsari ◽  
Ebrahim Talebi

Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder that typically presents with rapid appearance of tender skin lesions accompanied by fever and leukocytosis with neutrophilia. Its pathogenesis is not fully understood. The syndrome is generally classified into classical, malignancy-associated, and drug-induced categories, each of which has its specific characteristics. In this article, we present a case of classical Sweet’s syndrome in a woman who presented with an acute viral illness.


2016 ◽  
Vol 21 (1) ◽  
pp. 72-74 ◽  
Author(s):  
Allison K. Cinats ◽  
Richard M. Haber

Autoimmune hepatitis is a subtle diagnosis that has many diverse clinical presentations. It has been reported in the literature to occur concomitantly with pyoderma gangrenosum, a neutrophilic dermatosis. Sweet’s syndrome is another neutrophilic dermatosis and has been reported to be associated with autoimmune hepatitis in only 2 previous cases: 1 idiopathic and 1 drug induced. Here we report a third case in a 24-year-old woman diagnosed with Sweet’s syndrome in association with autoimmune hepatitis, documenting a possible trend between neutrophilic dermatoses and autoimmune hepatitis. The patient presented with a history of fever and tender, erythematous plaques on her legs. Skin biopsy of a plaque confirmed histiocytoid Sweet’s syndrome. Initial laboratory investigations revealed elevated transaminases, and liver biopsy confirmed autoimmune hepatitis. This case suggests autoimmune hepatitis should be considered as an association when investigating a patient with Sweet’s syndrome.


Author(s):  
Sukhjot Kaur ◽  
Amit Kumar Dhiman ◽  
Sandeep Kaur ◽  
Vikram Narang ◽  
Sunil Kumar Gupta ◽  
...  

<p class="abstract"><strong>Background:</strong> Sweet's syndrome is characterized by sudden onset of tender, erythematous papules, nodules, and plaques with a characteristic histopathology. It arises in three clinical settings- idiopathic, drug induced and malignancy induced. It has several clinical and histopathologic variants. Newer entities have been recently described in literature, thereby, emphasizing the need for continuous research. So, this study was conducted to add to scant data published in Indian literature with respect to this uncommon disease. The aims of the study were to bring insights into this rarely seen condition with special emphasis on histopathology; to highlight association of Sweet's syndrome with other systemic diseases, and its extra-cutaneous manifestations.</p><p class="abstract"><strong>Methods:</strong> A retrospective analysis of medical records from January 2013 to February 2017 of patients with Sweet’s syndrome was done in our dermatology department in a tertiary care hospital.<strong></strong></p><p class="abstract"><strong>Results:</strong> Out of total 13 patients, 6 (46%) were males and 7 (54%) were females. The age of patients ranged from 33 to 82 years. Five (39%) had idiopathic Sweet's syndrome, while 8 (61%) had non-idiopathic Sweet's syndrome, out of which three were associated with autoimmune diseases (ulcerative colitis, interstitial lung disease and crohn’s disease in 1 patient each), 3 cases were associated with malignancy (chronic neutrophilic leukemia, chronic myeloid leukemia and acute myelogenous leukemia in 1 patient each) and 2 cases were linked with infection. Bullous Sweet's syndrome was seen in 2 (15%) while neutrophilic dermatosis of dorsal hands was diagnosed in 5 (39%) cases. Four patients developed uncommon extracutaneous manifestations of Sweet's syndrome. In addition to classical histopathological findings of Sweet's syndrome, one case showed presence of immature neutrophils (histiocytoid Sweet's syndrome), fibrinoid necrosis was evident in 2 (15.4%), leucocytoclasia in 6 (46.2%), RBC extravasation in 3 (23.1%) and neutrophilic infiltration into vessel wall in 3 (23.1%) patients.</p><p><strong>Conclusions:</strong> Several unique variants were observed in the current study.</p>


2017 ◽  
Vol 4 (3) ◽  
pp. 847
Author(s):  
Hanock Unni Samuel ◽  
N. K. Thulaseedharan

Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that is typically located in the upper dermis. Sweet's syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. Classical Sweet's syndrome (CSS) usually presents in women between the age of 30 to 50 years, is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. We report here a case of classical sweets syndrome with the typical histopathological findings who reported well to treatment.


2015 ◽  
pp. 113-119
Author(s):  
Philip R. Cohen

Background: Sweet’s syndrome, also referred to as acute febrile neutrophilic dermatosis, can either occur as an idiopathic disorder or associated with another condition, including cancer, or induced by exposure to a drug. Proton pump inhibitors selectively inhibit gastric parietal cell H+-K+-adenosine triphosphatase and are most commonly used for the treatment of gastroesophageal reflux disease. Purpose: Proton pump inhibitor-associated Sweet’s syndrome is described in a woman with recurrent breast cancer. Methods: PubMed was used to search the following terms, separately and in combination: acute febrile neutrophilic dermatosis, breast cancer, malignancy, paraneoplastic, proton pump inhibitor, and Sweet’s syndrome. All papers were reviewed and relevant manuscripts, along with their reference citations, were evaluated. Results: Proton pump inhibitors have previously been associated with cutaneous adverse reactions including maculopapular rash, subacute cutaneous lupus erythematosus and toxic epidermal necrolysis. However, drug-induced Sweet’s syndrome has not been observed in patients receiving proton pump inhibitors. The reported woman developed Sweet’s syndrome after initial exposure and subsequent repeat challenge to proton pump inhibitors; subsequent studies also observed recurrence of her breast cancer presenting as metastases to her stomach and bone. Conclusions: Drug-induced Sweet’s syndrome has most commonly been associated with granulocyte colony stimulating factor in oncology patients. Malignancy-associated Sweet’s syndrome has been observed in patients with solid tumors, including breast cancer. Confirmation of proton pump inhibitor-induced Sweet’s syndrome, by repeat challenge with another medication in the same class of drug, was observed in a woman with breast cancer; although the subsequent discovery of recurrent breast cancer presenting as gastric mucosa and vertebral metastases also raises the possibility of concurrent paraneoplastic Sweet’s syndrome, her Sweet’s syndrome symptoms and lesions resolved without recurrence while her recurrent metastatic visceral malignancy persisted. In summary, medication-associated Sweet’s syndrome can occur in oncology patients and proton pump inhibitors should be added to the list of medications associated with the potential to cause drug-induced Sweet’s syndrome.


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