Case Report of Sweet’s Syndrome Associated With Autoimmune Hepatitis

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.

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.


2021 ◽  
Vol 12 (e) ◽  
pp. 1-4
Author(s):  
Ashwini Kodigehalli Chikkanna Swamy ◽  
Sandhaya Prasad ◽  
Anjan Kumar Patra

Sweet syndrome is an inflammatory dermatosis characterized by non-itchy, sometimes tender, erythematous plaques and papules most commonly distributed on the arms, upper body, head and neck. Histological findings include a dense dermal neutrophilic infiltrate with oedema. We report a case of 32year old female patient with solitary well defined erythematous edematous plaques with whitish scales at the centre of the lesion present over the extensor aspect of left forearm since 5days with sparing of palms , soles and mucosae. Laboratory investigations and histopathology were suggestive of Neutrophilic dermatoses (Sweet’s syndrome). This case was presented to highlight Atypical presentationof sweet syndrome – annular morphology and localization of lesions only over left forearm Therefore high index of suspicion is needed for its diagnosis in cases of solitary and isolated presentation along with necessary histopathological and laboratory investigations.


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.


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.


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>


2019 ◽  
Vol 3 (6) ◽  
pp. 431-434
Author(s):  
Maulik Manharlal Dhandha ◽  
Markiza Cooper ◽  
M Yadira Hurley ◽  
Sofia B Chaudhry

Autoimmunity Related Neutrophilic Dermatoses (ARND) is a recently described entity characterized by urticarial papules and plaques with histology showing a neutrophilic perivascular and interstitial infiltrates with leukocytoclasis, along with variable vacuolar interface dermatitis.  We report a 38 year old Caucasian female with a ten year history of lupus who presented with pruritic urticarial papules and plaques on the face, trunk, upper extremities and thighs.  The onset occurred 10 days after she started a calcium channel blocker (CCB), diltiazem. Histopathology revealed scattered dyskeratotic keratinocytes, vacuolar interface dermatitis, and a sparse perivascular and interstitial mixed infiltrate of neutrophils within the dermis. There was focal leukocytoclasis, dermal edema, and rare eosinophils.  The patient initially improved with prednisone taper, but flared again upon starting a different CCB, verapamil.  Exposure to certain medications such as CCB in the setting of autoimmune connective tissue disorder may be the inciting trigger for a neutrophilic dermatoses. Future studies may provide further information on the pathogenesis of ARND and cutaneous drug eruption in the setting of SLE.


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.


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