Sweet Syndrome: A Review and Update

Villarreal-Villarreal, César Daniel; Ocampo‐Candiani, Jorge; Villarreal‐Martínez, Alejandra

doi:10.1016/j.ad.2015.12.001

Cited by 108 publications

(109 citation statements)

References 81 publications

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“…A review of the literature shows that – irrespective of underlying etiology or association – two main features have been consistently present in all reported SS cases since the first description by Sweet in 1964, including the patients in our study cohort . The first criterion is the clinical presentation of “abrupt onset of painful or tender erythematous papules, plaques, or nodules”.…”

Section: Discussionmentioning

confidence: 55%

“…Besides, systemic corticosteroids are associated with well‐known hazards that make their use without confirmed diagnosis inadvisable. Furthermore, a number of recent reports showed an absent or poor response to systemic corticosteroids in some cases of SS (steroid‐refractory SS); here, some authors described an excellent response to other drugs such as colchicine . Therefore, we argue against including this item as “a minor criterion” for the diagnosis of SS, which has to be established prior to treatment initiation.…”

Section: Discussionmentioning

confidence: 90%

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Sweet's syndrome: diagnostic criteria revisited

Nofal

Abdelmaksoud

Amer³

et al. 2017

J Deutsche Derma Gesell

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Summary The diagnosis of Sweet's syndrome (SS) is based on a set of criteria that requires the presence of two major and at least two minor criteria. In some cases, however, the diagnosis is not as straightforward due to the absence of certain criteria. The objective of the present study was to review the clinical, histopathological, and laboratory features of the current diagnostic criteria for SS, and to evaluate their validity in the cases reported in the literature as well as in 40 patients treated at our institution. Our comprehensive review of the current criteria for SS reveals that the two major criteria have been consistently present in all cases – including ours – since the first description of SS in 1964. With regard to the minor criteria, on the other hand, there has been marked variability between different studies, and many cases failed to fulfill the requirement of showing two minor criteria. In order to simplify the diagnosis, avoid misdiagnosis, and allow for prompt treatment, we propose two sets of revised diagnostic criteria for SS. The first set comprises constant clinical and histopathological features that must be present and are by themselves sufficient for the diagnosis of SS to be established. The second set includes variable features whose absence does not warrant ruling out SS.

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Section: Discussionmentioning

confidence: 55%

Section: Discussionmentioning

confidence: 90%

Sweet's syndrome: diagnostic criteria revisited

Nofal

Abdelmaksoud

Amer³

et al. 2017

J Deutsche Derma Gesell

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“…Eine Überprüfung der Literatur zeigt, dass – unabhängig von der zugrunde liegenden Ätiologie oder Assoziation – seit der Erstbeschreibung durch Sweet im Jahr 1964 zwei Hauptmerkmale bei allen gemeldeten Fällen eines SS immer vorhanden waren, einschließlich der Patienten in unserer Studienkohorte . Das erste Kriterium ist das klinische Bild „plötzlich auftretender schmerzhafter oder empfindlicher erythematöser Papeln, Plaques und Knötchen“.…”

Section: Diskussionunclassified

“…Sie sollten daher ohne bestätigte Diagnose nicht verabreicht werden. Außerdem zeigten mehrere neuere Berichte bei einigen Fällen eines SS kein oder nur ein geringes Ansprechen auf systemische Kortikosteroide (steroidrefraktäres SS); in diesen Fällen beschrieben einige Autoren ein hervorragendes Ansprechen auf andere Medikamente, zum Beispiel Colchicin . Daher sind wir gegen die Einbeziehung dieses Punktes als „Nebenkriterium“ für die Diagnose eines SS, da diese vor Beginn der Behandlung gestellt werden muss.…”

Section: Diskussionunclassified

Sweet‐Syndrom: Revision der diagnostischen Kriterien

Nofal

Abdelmaksoud²,

Amer³

et al. 2017

J Deutsche Derma Gesell

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“…In contrast to SchS, CAPS is an autosomal dominant disorder with onset in early childhood ( 5 ). Another clinical differential diagnosis is Sweet syndrome, which, in contrast to SchS, rather shows erythematous nodules or plaques than urticarial rash, affects predominantly middle-aged women, is often malignancy-associated or drug-induced, and usually responds well to corticosteroids ( 10 ).…”

Section: Introductionmentioning

confidence: 99%

Case Report: Extensive Phosphorylation of Interleukin-1 Receptor-Associated Kinase 4 in a Patient With Schnitzler Syndrome

et al. 2020

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Schnitzler syndrome (SchS) is a rare autoinflammatory disease, characterized by urticarial rash, recurrent fever, osteo-articular pain/arthritis with bone condensation, and monoclonal gammopathy. Diagnosis may be difficult due to overlapping signs with other diseases. Here, we describe the case of a 62-year-old man with SchS, who was initially misdiagnosed with multicentric Castleman disease (MCD). As excessive release of IL-6 is characteristic of MCD, in contrast to IL-1 in SchS, we measured the phosphorylation of intracellular signaling proteins of the respective pathways by flow cytometry. We found a distinct increase of phosphorylated IRAK-4 in our patient’s B cells and monocytes while phosphorylation of STAT-3 was low, suggesting predominant IL-1 signaling. In accordance with these results and the classification criteria, we established the diagnosis of SchS instead of MCD and commenced therapy with the IL-1 receptor antagonist anakinra. We observed a rapid remission of signs accompanied by a reduction of phosphorylated IRAK-4 to normal levels. In conclusion, we propose phosphorylated IRAK-4 in B cells and monocytes as a potential marker for diagnosis of SchS and for treatment response to IL-1 blockade.

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Sweet Syndrome: A Review and Update

Cited by 108 publications

References 81 publications

Sweet's syndrome: diagnostic criteria revisited

Sweet's syndrome: diagnostic criteria revisited

Sweet‐Syndrom: Revision der diagnostischen Kriterien

Case Report: Extensive Phosphorylation of Interleukin-1 Receptor-Associated Kinase 4 in a Patient With Schnitzler Syndrome

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