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Miscellaneous Causes Of Unexplained Fever
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
We do not know the exact nosologic situation of these cases. They could be linked with the well-known various aspects of streptococcal allergies. For example, those presenting like “serum sickness”,19 cutaneous vasculitis,20 or, even more disturbing, polyarteritis nodosa.21 Clinically, they are not very different from adult Still’s disease (see Chapter 18). They also have common features with the acute febrile neutrophilic dermatosis of Sweet.22 They are very close, if not identical, to subsepsis allergica of Wissler.23
Haematology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Sweet syndrome (SS) or acute febrile neutrophilic dermatosis is chracterised by fever, painful erythematous papules, nodules and plaques usually over the trunk, face and upper limbs usually in association with a neutrophil leucocytosis. It can present in several clinical settings: classical (idiopathic) SS, malignancy-associated SS, and drug-induced SS, especially following G-CSF administration.
Diagnosis of IBD
Published in Peter Sagar, Andrew G. Hill, Charles H. Knowles, Stefan Post, Willem A. Bemelman, Patricia L. Roberts, Susan Galandiuk, John R.T. Monson, Michael R.B. Keighley, Norman S. Williams, Keighley & Williams’ Surgery of the Anus, Rectum and Colon, 2019
Gregor Novak, Geert D’Haens, Najib Haboubi, John B. Schofield
Sweet’s syndrome (acute febrile neutrophilic dermatosis) is a rare dermatologic EIM of IBD which is associated with active disease. It manifests as tender papulosquamous exanthema or nodules involving the arm, legs, trunk, hands or face.127 Anti-TNF treatment can induce paradoxical inflammation of the skin which is usually reversible upon drug cessation. Skin lesions (psoriasiform eczema, eczema and xerosis) are reported in approximately 22% of patients treated on anti-TNF therapy.132
An update on VEXAS syndrome
Published in Expert Review of Clinical Immunology, 2023
Common inflammatory phenotypes seen in VEXAS include Sweet’s syndrome, relapsing polychondritis (RP) and polyarteritis nodosa (PAN). Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, presents with fever, neutrophilia and cutaneous or mucosal lesions. The characteristic lesions are tender erythematous papules, nodules or plaques which, on biopsy, show an infiltrate of mostly mature neutrophils [44]. In a study of eight patients with VEXAS and neutrophilic dermatosis, histology showed infiltrates of mature neutrophils with myeloid and lymphoid cells containing the same UBA1 mutation as paired bone marrow samples in all patients, suggesting the infiltrates were derived from the pathological clone [45]. Interestingly, in another study, this finding was replicated in two biopsies showing neutrophilic dermatoses but the UBA1 mutation was not identified in cells from leukocytoclastic vasculitis (LCV) (n = 2) or septal panniculitis (n = 2) suggesting a differentiation of ‘clonal’ (neutrophilic dermatoses) and ‘paraclonal’ (LCV and septal panniculitis) cutaneous manifestations of the disease, which might help to direct targeted therapies in the future [46].
Histiocytoid Sweet syndrome successfully treated with etanercept
Published in Baylor University Medical Center Proceedings, 2018
Ian T. Watson, Isabel Haugh, Alexis R. Gardner, M. Alan Menter
Acute febrile neutrophilic dermatosis, previously called Sweet syndrome, is an uncommon condition characterized by fever, elevated neutrophil count, and tender, red, cutaneous papules, plaques, or nodules.1 Histiocytoid Sweet syndrome (HSS) is a rare histological variant of acute febrile neutrophilic dermatosis, which is distinguished by dermal infiltration with histiocytoid mononuclear cells, thought to be immature neutrophils.2 Etanercept is a recombinant fusion protein that inhibits tumor necrosis factor-α (TNF-α), a cytokine that acts on several cell types to promote inflammation.3–5 Here, we present a case of a 34-year-old woman with HSS successfully treated with etanercept with a review of pertinent earlier publications.
Multiple painful plaques and the Sweet’s syndrome
Published in Baylor University Medical Center Proceedings, 2022
Debra Lee, Daniel Baird, Michelle Tarbox
Acute febrile neutrophilic dermatosis, also known as Sweet’s syndrome (SS), traditionally manifests as acute onset fever, neutrophilia, and painful cutaneous lesions. These lesions appear as purple-red papules and plaques that are often distributed asymmetrically across the face, neck, and upper extremities. SS is characterized histologically by a diffuse infiltrate predominantly composed of mature neutrophils in the dermis. It is a rare inflammatory skin disease of unknown origin, associated with a multitude of diseases, infections, malignancies, and medications.