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Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, [1] [2] is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
The current (2008) diagnostic criteria for HLH are [23] 1. A molecular diagnosis consistent with HLH. These include the identification of pathologic mutations of PRF1, UNC13D, or STX11. OR 2. Fulfillment of five out of the eight criteria below: Fever (defined as a temperature >100.3 °F, >38 °C) Enlargement of the spleen
Sweet's syndrome-like dermatosis is a cutaneous condition associated with bowel disorders. [1] See also. Sweet's syndrome; List of cutaneous conditions; References
Sweet disease (or acute febrile neutrophilic dermatosis) is a systemic inflammatory disorder characterized by fever, peripheral neutrophilic leukocytosis, and neutrophilic infiltrates in the skin, among other symptoms. [17] When complicated by encephalitis or meningitis, it is referred to as neuro-Sweet disease.
Chronic recurrent multifocal osteomyelitis (CRMO) is a rare condition (1:1,000,000), in which the bones have lesions, inflammation, and pain.It is called multifocal because it can appear in different parts of the body, primarily bones, and osteomyelitis because it is very similar to that disease, although CRMO appears to be without any infection.
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In addition to a biopsy demonstrating a neutrophilic infiltrate, patients must have at least 4 minor criteria to meet diagnostic criteria. [17] These criteria are based on histology, history, clinical examination and treatment. [citation needed] Histology: Exclusion of infection (including histologically indicated stains and tissue cultures)
As of 2017 the cause of the disease was not understood. [1] A 2024 review by Braud and Lipsker aimed to "describe what is currently known about the pathogenesis of this peculiar disease, as well as to address its diagnosis and management" and concluded that "physiopathology of Schnitzler syndrome remains elusive" and "the main question regarding the relationship between the autoinflammatory ...