Sweet's syndrome in a patient with chronic lymphocytic leukaemia

Smith, Catherine R.; Williams, Penelope

doi:10.1136/bcr-2017-220317

Cited by 3 publications

(3 citation statements)

References 12 publications

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“…In CLL, accounting for approximately 6% of all SS, skin lesions often appear on the face and scalp and seem more persistent. Our case is the first description of a CLL‐associated nSS, although the influenza infection might have acted as an additional trigger 3,4 . The underlying pathomechanisms of SS are not fully understood.…”

Section: Figurementioning

confidence: 90%

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A diagnostic challenge—First case of chronic lymphatic leukemia‐associated necrotizing sweet syndrome

Neumann,

Nieper,

Simon

et al. 2023

European J of Haematology

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Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder typically characterized by the clinical triad including a sudden onset of fever, painful skin lesions, and neutrophilia. The histopathological findings are a dense neutrophilic infiltrate and oedema of the dermis and epidermis without evidence of a vasculitis. Besides treatment of the underlying cause, sweet syndrome is typically treated with high‐dose corticosteroids leading to a relapse‐free response in 70% of patients. However, if left unrecognized or untreated, the condition may lead to serious complications. Here, we report on the case of a 38‐year‐old patient in whom, under the assumption of the presence of necrotizing fasciitis, exarticulation of the right arm was performed. In the absence of pathogen detection and insufficient response to anti‐infective therapies, the diagnosis of a sweet syndrome was assumed and, later, confirmed by an excellent response to high‐dose administration of systematic glucocorticoids. The case emphasizes the need to be aware of this rare syndrome, which can be easily misdiagnosed due to its close resemblance to infection and stresses the need of further research to define distinct diagnostic tools.

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Section: Figurementioning

confidence: 90%

“…Our case is the first description of a CLL-associated nSS, although the influenza infection might have acted as an additional trigger. 3,4 The underlying pathomechanisms of SS are not fully understood.…”

mentioning

confidence: 99%

A diagnostic challenge—First case of chronic lymphatic leukemia‐associated necrotizing sweet syndrome

Neumann,

Nieper,

Simon

et al. 2023

European J of Haematology

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“…A Síndrome de Sweet foi originalmente descrita pelo Dr. Robert Sweet em 1964, como "Dermatose Neutrofílica febril aguda", tendo como base a apresentação clínica-patológica de 8 mulheres que apresentavam febre de início agudo, placas eritematosas sensíveis com infiltração neutrofílica densa na derme e leucocitose (Smith & Williams, 2017). Essas mulheres não apresentavam evidências de infecções e apresentavam respostas rápida ao uso dos corticosteroides (Vithoosan et al, 2019).…”

Section: Introductionunclassified

Síndrome de Sweet: relato de caso de um acontecimento raro

Leite

Santos

Silva

et al. 2021

RSD

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A síndrome de Sweet (SS) e uma condição inflamatória sistêmica rara, que se caracteriza-se pelo aparecimento súbito de placas e nódulos cutâneos eritêmo-edematoso, relacionados a sintomas sistêmicos e a infiltração de neutrófilos maduros a estudos histopatológicos. Atualmente a doença é classificada em três categorias: clássica (idiopática), associada à malignidade e induzida por drogas, sendo que a clássica constitui a maioria dos casos. Nesse contexto, apresentamos um caso de um homem de 72 anos com quadro típico da forma clássica de Síndrome de Sweet, onde apresentou regressão das lesões após 2 semanas de tratamento, sendo que nesses casos a regressão inicia-se 72 horas após o início da corticoterapia sistêmica. Apesar da Síndrome de Sweet ser uma doença rara, com o diagnóstico e a instauração terapêutica adequada e com a utilização da corticoterapia sistêmica, os resultados acabam sendo extremamente rápidos e favoráveis aos seus portadores. Assim sendo, o diagnóstico e a classificação da forma da Síndrome de Sweet concede um direcionamento terapêutico adequado, tendo em vista que nos casos em que são induzidos por drogas ou associados a neoplasias, só existem respostas terapêuticas satisfatórias e duradouras, quando se realiza o tratamento da doença de base e a suspensão da droga causadora, respectivamente.

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Insights Into the Pathogenesis of Sweet's Syndrome

2019

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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 classic Sweet's syndrome, malignancy associated, and drug induced. 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.

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Sweet's syndrome in a patient with chronic lymphocytic leukaemia

Cited by 3 publications

References 12 publications

A diagnostic challenge—First case of chronic lymphatic leukemia‐associated necrotizing sweet syndrome

A diagnostic challenge—First case of chronic lymphatic leukemia‐associated necrotizing sweet syndrome

Síndrome de Sweet: relato de caso de um acontecimento raro

Insights Into the Pathogenesis of Sweet's Syndrome

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