New Practical Aspects of Sweet Syndrome

Authors

Tejas P Joshi
Sarah K Friske
David A Hsiou
Madeleine Duvic

Publication Date

5-1-2022

Journal

American Journal of Clinical Dermatology

Abstract

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.

Keywords

Adrenal Cortex Hormones, Humans, Iatrogenic Disease, Neck, Skin, Sweet Syndrome

DOI

10.1007/s40257-022-00673-4

PMID

35157247

PMCID

PMC8853033

PubMedCentral® Posted Date

2-14-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes