Necrotising Sweet’s syndrome: a rare presentation of Sweet’s syndrome with unilateral painful swelling of the neck

Husted, Andreas; Udholm, Sebastian; Udholm, Nichlas

doi:10.1136/bcr-2021-244179

Cited by 2 publications

(1 citation statement)

References 17 publications

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“…Differentiating NSS from NF is critical since the treatment is fundamentally different. NSS is often diagnosed after repeated negative wound cultures and worsening of disease despite surgical debridement and antibiotics, as was seen in the case described here [5, 6]. NSS clinically and histopathologically resembles NF, with features of fat necrosis and myonecrosis, but the lesions occur in the absence of an infectious cause.…”

Section: Discussionmentioning

confidence: 99%

A Case of Acute Myeloid Leukemia-Associated Necrotizing Sweet Syndrome

et al. 2022

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Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a rare painful skin condition that is characterized by hyperpyrexia, peripheral blood and skin neutrophilia, and edematous skin lesions. Necrotizing SS (NSS) is a severe and locally aggressive condition that histopathologically resembles a necrotizing soft tissue infection. As opposed to necrotizing soft tissue infections, NSS responds to systemic steroids. SS is divided into three subtypes: classical SS, malignancy-associated SS, and drug-induced SS. Within the malignancy-associated SS subtype, both solid tumor and hematologic malignancies have been precursors to developing SS. Here, we present a case of acute myeloid leukemia-associated NSS.

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Section: Discussionmentioning

confidence: 99%

A Case of Acute Myeloid Leukemia-Associated Necrotizing Sweet Syndrome

et al. 2022

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New Practical Aspects of Sweet Syndrome

et al. 2022

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Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous papules/plaques/pustules/nodules commonly appearing on the upper limbs, trunk, and head and neck; histologically, SS is characterized by dense neutrophilic infiltrate in the dermis. SS is accompanied by fever; an elevation of inflammatory markers (e.g., erythrocyte sedimentation rate, C reactive protein) in serum may also be observed. Although most cases of SS are idiopathic, SS also occurs in the setting of malignancy or following administration of an associated drug. SS has also been reported in association with pregnancy and a burgeoning list of infectious (most commonly upper respiratory tract infections) and inflammatory diseases; likewise, the litany of possible iatrogenic triggers has also grown. Over the past several years, a wider spectrum of SS presentation has been realized, with several reports highlighting novel clinical and histological variants. Corticosteroids continue to be efficacious first-line therapy for the majority of patients with SS, although novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory SS. New mechanisms of SS induction have also been recognized, although the precise etiology of SS still remains elusive. Here, we catalogue the various clinical and histological presentations of SS, summarize recently reported disease associations and iatrogenic triggers, and review treatment options. We also attempt to frame the findings of this review in the context of established and emerging paradigms of SS pathogenesis.

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Necrotising Sweet’s syndrome: a rare presentation of Sweet’s syndrome with unilateral painful swelling of the neck

Cited by 2 publications

References 17 publications

A Case of Acute Myeloid Leukemia-Associated Necrotizing Sweet Syndrome

A Case of Acute Myeloid Leukemia-Associated Necrotizing Sweet Syndrome

New Practical Aspects of Sweet Syndrome

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