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Frontiers in Immunology 2019Sweet's syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare inflammatory condition. It is considered to be the prototype disease of neutrophilic... (Review)
Review
Sweet's syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare inflammatory condition. It is considered to be the prototype disease of neutrophilic dermatoses, and presents with acute onset dermal neutrophilic lesions, leukocytosis, and pyrexia. Several variants have been described both clinically and histopathologically. Classifications include . The cellular and molecular mechanisms involved in Sweet's syndrome have been difficult to elucidate due to the large variety of conditions leading to a common clinical presentation. The exact pathogenesis of Sweet's syndrome is unclear; however, new discoveries have shed light on the role of inflammatory signaling, disease induction, and relationship with malignancy. These findings include an improved understanding of inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic contributions. Continued investigations into effective treatments and targeted therapy will benefit patients and improve our molecular understanding of inflammatory diseases, including Sweet's syndrome.
Topics: Dermatitis; Female; Humans; Inflammasomes; Interleukin-17; Interleukin-1beta; Neutrophil Infiltration; Neutrophils; Rare Diseases; Sweet Syndrome
PubMed: 30930894
DOI: 10.3389/fimmu.2019.00414 -
Anais Brasileiros de Dermatologia 2018Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory...
Sweet's syndrome is a rare dermatosis with little-known pathogenesis, associated with some clinical conditions such as infections, autoimmune diseases, inflammatory bowel diseases, vaccination, medications and neoplasms. Hematologic malignancies are the diseases most related to paraneoplastic Sweet's syndrome, but this clinical entity can also be found occasionally in some solid tumors, including genitourinary tract tumors. We report a rare case of paraneoplastic Sweet's syndrome associated with the diagnosis of cervical cancer.
Topics: Diagnosis, Differential; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Paraneoplastic Syndromes; Sweet Syndrome; Uterine Cervical Neoplasms
PubMed: 30066769
DOI: 10.1590/abd1806-4841.20187353 -
International Journal of... Jun 2016Sweet's syndrome, or acute febrile neutrophilic dermatosis, is an uncommon severe cutaneous condition, not previously associated with allopurinol therapy. We describe...
Sweet's syndrome, or acute febrile neutrophilic dermatosis, is an uncommon severe cutaneous condition, not previously associated with allopurinol therapy. We describe the case of an 87-year-old woman with hyperuricemia who developed classic Sweet's syndrome manifestations 8 days after being treated with allopurinol. Patient's symptoms included fever, painful edema in the hands and lower limbs with non-pruritic erythematous plaques topped by pus-filled skin blisters, right eye conjunctivitis, splenomegaly and joint pain. At the emergency department, blood tests showed neutrophilic leukocytosis, inflammatory state and altered liver function. During hospitalization, she received unsuccessful treatments with two different antibiotics (namely ceftriaxone and levofloxacin), while treatment with intravenous methylprednisolone produced a rapid clinical remission of symptoms, cutaneous lesion pain improvement, normalization of her body temperature and her blood values returned to normal. Use of the Naranjo adverse drug reaction probability scale indicated a probable relationship between the patient's development of Sweet's syndrome and allopurinol therapy. Because the signs and symptoms of Sweet's syndrome resemble an infectious process, the correct diagnosis may be delayed and inappropriate treatment regimen with antibiotics may often precede glucocorticoid therapy.
Topics: Aged, 80 and over; Allopurinol; Anti-Bacterial Agents; Female; Humans; Hyperuricemia; Sweet Syndrome
PubMed: 26684631
DOI: 10.1177/0394632015599705 -
Indian Journal of Ophthalmology Sep 2020Ocular manifestations of Sweet syndrome, or acute febrile neutrophilic dermatosis, are usually limited to the anterior segment. We report the case of a patient with...
Ocular manifestations of Sweet syndrome, or acute febrile neutrophilic dermatosis, are usually limited to the anterior segment. We report the case of a patient with bilateral panuveitis and retinal vasculitis associated with Sweet syndrome. A 45-year-old Asian female with an undiagnosed febrile illness with rash presented with bilateral panuveitis with haemorrhagic occlusive retinal vasculitis. Skin biopsy confirmed Sweet Syndrome. Intraocular inflammation resolved with a combination of topical and systemic corticosteroids as well as intravenous cyclophosphamide, with resulting permanent severe right visual impairment. Although an uncommon condition, Sweet syndrome should be considered in any febrile patient with skin lesions and uveitis.
Topics: Biopsy; Female; Humans; Middle Aged; Panuveitis; Retinal Vasculitis; Skin; Sweet Syndrome
PubMed: 32823459
DOI: 10.4103/ijo.IJO_1667_19 -
Life (Basel, Switzerland) Mar 2023Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The... (Review)
Review
Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients.
PubMed: 36983964
DOI: 10.3390/life13030809 -
Proceedings (Baylor University. Medical... 2024
PubMed: 38174001
DOI: 10.1080/08998280.2023.2282859 -
Proceedings (Baylor University. Medical... 2022We present a rare case of Sweet's syndrome. A 39-year-old woman with subjective fevers, polyarthralgia, and malaise presented with worsening painful erythematous plaques...
We present a rare case of Sweet's syndrome. A 39-year-old woman with subjective fevers, polyarthralgia, and malaise presented with worsening painful erythematous plaques on the trunk, arms, and legs. Further examination with biopsy revealed a diagnosis of acute febrile neutrophilic dermatosis, or Sweet's syndrome. Diagnosis by skin biopsy is crucial, and onset requires prompt evaluation for serious associated disorders such as leukemias, inflammatory bowel disease, thyroid disease, sarcoidosis, and infectious etiologies. In general, symptoms and cutaneous manifestations of Sweet syndrome respond rapidly to treatment with systemic corticosteroids or potassium iodide.
PubMed: 34970040
DOI: 10.1080/08998280.2021.1980307 -
CMAJ : Canadian Medical Association... Jul 2023
Topics: Humans; Sweet Syndrome; Neoplasms
PubMed: 37524395
DOI: 10.1503/cmaj.220827-f -
Current Health Sciences Journal 2020Sweet syndrome (SS), also denominated as acute febrile neutrophilic dermatosis, is a rare disease characterized by the sudden onset of painful, erythematous, firm skin...
INTRODUCTION
Sweet syndrome (SS), also denominated as acute febrile neutrophilic dermatosis, is a rare disease characterized by the sudden onset of painful, erythematous, firm skin lesions (papules, plaques, and nodules) which show, upon histologic examination, the presence of a diffuse infiltrate of mature neutrophils. The cutaneous manifestation typically involves the face, neck, trunk, and upper limbs and is associated with fever, general malaise, arthralgia.
CASE REPORT
A female patient, 60 years old, attended the Dermatology Clinic due to the appearance of violaceous erythematous-oedematous infiltrated plaques, located on the face, neck, upper limbs, trunk and knees. The onset of the cutaneous manifestation had occurred 2 months prior, accompanied by pain, chills, flares of fever and arthralgia. The onset coincided with the surgical treatment of an umbilical hernia. From the medical history we note that the patient was diagnosed in 2014 with histiocytoid SS. She followed a treatment with methylprednisolone, with positive response, but had many relapses after the discontinuation of treatment. In 2017, due to a new episode, the histopathological examination was repeated, which revealed classical SS. She received treatment with Disulone and Colchicine. She had not been administered any treatment throughout the previous year. Laboratory tests revealed leukocytosis with neutrophils, increased ESR, elevated C4, hyperglycemia. The current histopathological examination revealed lymphocytic SS. Under treatment with methylprednisolone 32mg/day, the evolution was favorable.
DISCUSSIONS
The first case of SS was described by Robert Douglas Sweet in 1964. As known aetiological factors there have been described gastrointestinal and urinary tract infections, pregnancy, inflammatory bowel disease, drugs or malignancies. There have been described cases of SS that appeared after surgical treatment, as in our case, which registered a new outbreak following the umbilical hernia treatment. The histopathological variants of SS described in the literature are: subcutaneous, eosinophilic, histiocytoid, lymphocytic type. The first line-therapy consists in systemic corticosteroids, which induce a fast remission of lesions and general symptoms. Recurrence may occur in approximately 50% of patients and is common in idiopathic or paraneoplastic cases.
CONCLUSIONS
In addition to the neutrophilic infiltrate that is typical for Sweet syndrome, different types of histological manifestations have been described in the literature: subcutaneous, eosinophilic, histiocytoid, lymphocytic. In our case, we noted that the histological profile changed over time, from a histiocytoid SS recorded in 2014, to a classical SS in 2017, followed by the appearance of lymphocytic SS in 2019. Due to the fact that SS can be associated with a numerous other disorders, our patient requires regular monitoring with a view to eliminate them, and potentially to make a diagnosis and initiate early specific treatment.
PubMed: 32637170
DOI: 10.12865/CHSJ.46.01.12 -
The Australasian Journal of Dermatology Nov 2022We describe a strikingly robust presentation of trimethoprim-sulfamethoxazole (TMP-SMX)-induced pustular Sweet syndrome and discuss how to distinguish it from iododerma... (Review)
Review
We describe a strikingly robust presentation of trimethoprim-sulfamethoxazole (TMP-SMX)-induced pustular Sweet syndrome and discuss how to distinguish it from iododerma and other neutrophil-rich conditions. A review of the literature indicates that TMP-SMX-induced Sweet syndrome (SS) may have higher rates of neutrophilia and greater ocular, mucosal, and musculoskeletal involvement compared to SS from other drugs. Recognizing these features and identifying the offending agent are critical for correctly diagnosing TMP-SMX-induced SS in a timely manner.
Topics: Humans; Trimethoprim, Sulfamethoxazole Drug Combination; Sweet Syndrome
PubMed: 35866718
DOI: 10.1111/ajd.13897